Michael R. Hayden
FACULTY & STAFF DIRECTORY
ADDITIONAL CONTACT INFORMATION
RESEARCH INTERESTSPlease visit my lab website to learn more about my research interests and projects.PublicationsReviews and Invited Reviews1 Review Ehrnhoefer DE, Butland SL, Pouladi MA, Hayden MR. Mouse models of Huntington disease: variations on a theme. Dis Model Mech 2(3-4):123-9. (2009) PMID 19259385 2 Review Hayden MR, Leavitt BR, Yasothan U, Kirkpatrick P. Tetrabenazine. 8(1):17-8. (2009) PMID 19116624 3 Review Brunham LR, Kruit JK, Verchere CB, Hayden MR. Cholesterol in islet dysfunction and type 2 diabetes. J. Clin. Invest. 118(2):403-8. (2008) PMID 18246189 Journal Articles1 Sistonen J, Madadi P, Ross CJ, Yazdanpanah M, Lee JW, Landsmeer ML, Nauta M, Carleton BC, Koren G, Hayden MR. Prediction of codeine toxicity in infants and their mothers using a novel combination of maternal genetic markers. Clin. Pharmacol. Ther. 91(4):692-9. (2012) PMID 22398969 2 Lee JM, Ramos EM, Lee JH, Gillis T, Mysore JS, Hayden MR, Warby SC, Morrison P, Nance M, Ross CA, Margolis RL, Squitieri F, Orobello S, Di Donato S, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Abramson RK, Marder K, Sequeiros J, Paulsen JS, , Landwehrmeyer GB, , Myers RH, , Macdonald ME, Gusella JF, . CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion. 78(10):690-695. (2012) PMID 22323755 3 Wijesekara N, Zhang LH, Kang MH, Abraham T, Bhattacharjee A, Warnock GL, Verchere CB, Hayden MR. miR-33a Modulates ABCA1 Expression, Cholesterol Accumulation, and Insulin Secretion in Pancreatic Islets. Diabetes 61(3):653-8. (2012) PMID 22315319 4 Kruit JK, Wijesekara N, Westwell-Roper C, Vanmierlo T, de Haan W, Bhattacharjee A, Tang R, Wellington CL, Lütjohann D, Johnson JD, Brunham LR, Verchere CB, Hayden MR. Loss of Both ABCA1 and ABCG1 Results in Increased Disturbances in Islet Sterol Homeostasis, Inflammation, and Impaired β-Cell Function. Diabetes 61(3):659-64. (2012) PMID 22315310 5 Bombard Y, Palin J, Friedman JM, Veenstra G, Creighton S, Bottorff JL, Hayden MR, . Beyond the patient: the broader impact of genetic discrimination among individuals at risk of Huntington disease. Am. J. Med. Genet. B Neuropsychiatr. Genet. 159B(2):217-26. (2012) PMID 22231990 6 Waldron-Roby E, Ratovitski T, Wang X, Jiang M, Watkin E, Arbez N, Graham RK, Hayden MR, Hou Z, Mori S, Swing D, Pletnikov M, Duan W, Tessarollo L, Ross CA. Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin. J. Neurosci. 32(1):183-93. (2012) PMID 22219281 7 Goldberg YP, Price N, Namdari R, Cohen CJ, Lamers MH, Winters C, Price J, Young CE, Verschoof H, Sherrington R, Pimstone SN, Hayden MR. Treatment of Na(v)1.7-mediated pain in inherited erythromelalgia using a novel sodium channel blocker. Pain 153(1):80-5. (2012) PMID 22035805 8 van den Anker JN. Is it safe to use opioids for obstetric pain while breastfeeding? J. Pediatr. 160(1):4-6. (2012) PMID 21996159 9 Ketelaar M, Hofstra R, Hayden M. What monozygotic twins discordant for phenotype illustrate about mechanisms influencing genetic forms of neurodegeneration. Clin. Genet. 81(4):325-33. (2012) PMID 21981075 10 Franciosi S, Ryu JK, Shim Y, Hill A, Connolly C, Hayden MR, McLarnon JG, Leavitt BR. Age-dependent neurovascular abnormalities and altered microglial morphology in the YAC128 mouse model of Huntington disease. Neurobiol. Dis. 45(1):438-49. (2012) PMID 21946335 11 Lam J, Kelly L, Ciszkowski C, Landsmeer ML, Nauta M, Carleton BC, Hayden MR, Madadi P, Koren G. Central nervous system depression of neonates breastfed by mothers receiving oxycodone for postpartum analgesia. J. Pediatr. 160(1):33-7.e2. (2012) PMID 21880331 12 Tietjen I, Hovingh GK, Singaraja R, Radomski C, McEwen J, Chan E, Mattice M, Legendre A, Kastelein JJ, Hayden MR. Increased risk of coronary artery disease in Caucasians with extremely low HDL cholesterol due to mutations in ABCA1, APOA1, and LCAT. Biochim. Biophys. Acta 1821(3):416-24. (2012) PMID 21875686 13 Ehrnhoefer DE, Skotte NH, Savill J, Nguyen YT, Ladha S, Cao LP, Dullaghan E, Hayden MR. A quantitative method for the specific assessment of caspase-6 activity in cell culture. PLoS ONE 6(11):e27680. (2011) PMID 22140457 14 Graham RK, Ehrnhoefer DE, Hayden MR. Caspase-6 and neurodegeneration. Trends Neurosci. 34(12):646-56. (2011) PMID 22018804 15 Ehrnhoefer DE, Wong BK, Hayden MR. Convergent pathogenic pathways in Alzheimer's and Huntington's diseases: shared targets for drug development. Nat Rev Drug Discov 10(11):853-67. (2011) PMID 22015920 16 Kruit JK, Wijesekara N, Fox JE, Dai XQ, Brunham LR, Searle GJ, Morgan GP, Costin AJ, Tang R, Bhattacharjee A, Johnson JD, Light PE, Marsh BJ, Macdonald PE, Verchere CB, Hayden MR. Islet Cholesterol Accumulation Due to Loss of ABCA1 Leads to Impaired Exocytosis of Insulin Granules. Diabetes 60(12):3186-96. (2011) PMID 21998401 17 Ding Y, Zhang L, Wang Y, Huang W, Tang Y, Bai L, Ross CJ, Hayden MR, Liu G. Amelioration of hypertriglyceridemia with hypo-alpha-cholesterolemia in LPL deficient mice by hematopoietic cell-derived LPL. PLoS ONE 6(9):e25620. (2011) PMID 21980507 18 Carroll JB, Warby SC, Southwell AL, Doty CN, Greenlee S, Skotte N, Hung G, Bennett CF, Freier SM, Hayden MR. Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin. Mol. Ther. 19(12):2178-85. (2011) PMID 21971427 19 Hawkins AK, Ho A, Hayden MR. Lessons from predictive testing for Huntington disease: 25 years on. J. Med. Genet. 48(10):649-50. (2011) PMID 21931167 20 Karasinska JM, Hayden MR. Cholesterol metabolism in Huntington disease. Nat Rev Neurol 7(10):561-72. (2011) PMID 21894212 21 Carroll JB, Southwell AL, Graham RK, Lerch JP, Ehrnhoefer DE, Cao LP, Zhang WN, Deng Y, Bissada N, Henkelman RM, Hayden MR. Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease. Mol Neurodegener 6:59. (2011) PMID 21854568 22 Singaraja RR, Huang K, Sanders SS, Milnerwood AJ, Hines R, Lerch JP, Franciosi S, Drisdel RC, Vaid K, Young FB, Doty C, Wan J, Bissada N, Henkelman RM, Green WN, Davis NG, Raymond LA, Hayden MR. Altered palmitoylation and neuropathological deficits in mice lacking HIP14. Hum. Mol. Genet. 20(20):3899-909. (2011) PMID 21775500 23 VanderVaart S, Berger H, Sistonen J, Madadi P, Matok I, Gijsen VM, de Wildt SN, Taddio A, Ross CJ, Carleton BC, Hayden MR, Koren G. CYP2D6 polymorphisms and codeine analgesia in postpartum pain management: a pilot study. Ther Drug Monit 33(4):425-32. (2011) PMID 21743374 24 Joly Y, Koutrikas G, Ramos-Paque E, Zawati M, Gardy J, Hayden MR, Carleton BC. Diagnostic testing for vaccinomics: is the regulatory approval framework adequate? A comparison of Canada, the United States, and Europe. OMICS 15(9):597-605. (2011) PMID 21728814 25 Huang K, Sanders SS, Kang R, Carroll JB, Sutton L, Wan J, Singaraja R, Young FB, Liu L, El-Husseini A, Davis NG, Hayden MR. Wild-type HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14. Hum. Mol. Genet. 20(17):3356-65. (2011) PMID 21636527 26 Brunham L, Tietjen I, Bochem A, Singaraja R, Franchini P, Radomski C, Mattice M, Legendre A, Hovingh G, Kastelein J, Hayden M. Novel mutations in scavenger receptor BI associated with high HDL cholesterol in humans. Clin. Genet. 79(6):575-81. (2011) PMID 21480869 27 Madadi P, Joly Y, Avard D, Chitayat DC, Smith MA, D Ross CJ, Carleton BC, Hayden MR, Koren G. The communication of pharmacogenetic research results: participants weigh in on their informational needs in a pilot study. J Popul Ther Clin Pharmacol 18:e152-5. (2011) PMID 21467605 28 Ross CJ, Visscher H, Rassekh SR, Castro-Pastrana LI, Shereck E, Carleton B, Hayden MR. Pharmacogenomics of serious adverse drug reactions in pediatric oncology. J Popul Ther Clin Pharmacol 18:e134-51. (2011) PMID 21467604 29 Carroll JB, Lerch JP, Franciosi S, Spreeuw A, Bissada N, Henkelman RM, Hayden MR. Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease. Neurobiol. Dis. 43(1):257-65. (2011) PMID 21458571 30 Visscher H, Amstutz U, Sistonen J, Ross CJ, Hayden MR, Carleton BC. Pharmacogenomics of cardiovascular drugs and adverse effects in pediatrics. J. Cardiovasc. Pharmacol. 58(3):228-39. (2011) PMID 21386709 31 Song W, Chen J, Petrilli A, Liot G, Klinglmayr E, Zhou Y, Poquiz P, Tjong J, Pouladi MA, Hayden MR, Masliah E, Ellisman M, Rouiller I, Schwarzenbacher R, Bossy B, Perkins G, Bossy-Wetzel E. Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat. Med. 17(3):377-82. (2011) PMID 21336284 32 Ehrnhoefer DE, Sutton L, Hayden MR. Small changes, big impact: posttranslational modifications and function of huntingtin in Huntington disease. Neuroscientist 17(5):475-92. (2011) PMID 21311053 33 Hawkins AK, Hayden MR. A grand challenge: providing benefits of clinical genetics to those in need. Genet. Med. 13(3):197-200. (2011) PMID 21283011 34 Warby SC, Visscher H, Collins JA, Doty CN, Carter C, Butland SL, Hayden AR, Kanazawa I, Ross CJ, Hayden MR. HTT haplotypes contribute to differences in Huntington disease prevalence between Europe and East Asia. Eur. J. Hum. Genet. 19(5):561-6. (2011) PMID 21248742 35 Ye D, Zhao Y, Hildebrand RB, Singaraja RR, Hayden MR, Van Berkel TJ, Van Eck M. The dynamics of macrophage infiltration into the arterial wall during atherosclerotic lesion development in low-density lipoprotein receptor knockout mice. Am. J. Pathol. 178(1):413-22. (2011) PMID 21224078 36 Joly Y, Koutrikas G, Tassé A-M, Issa A, Carleton B, Hayden MR, Rieder MJ, Ramos-Paque E, Avard D. Regulatory Approval for New Pharmacogenomics Tests: A Comparative Overview. Food and Drug Law Journal (2011) 37 Bombard Y, Palin J, Friedman JM, Veenstra G, Creighton S, Paulsen JS, Bottorff JL, Hayden MR, . Factors associated with experiences of genetic discrimination among individuals at risk for huntington disease. 156(1):19-27. (2010) PMID 21069869 38 Graham RK, Deng Y, Carroll J, Vaid K, Cowan C, Pouladi MA, Metzler M, Bissada N, Wang L, Faull RL, Gray M, Yang XW, Raymond LA, Hayden MR. Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo. J. Neurosci. 30(45):15019-29. (2010) PMID 21068307 39 Sturrock A, Laule C, Decolongon J, Dar Santos R, Coleman AJ, Creighton S, Bechtel N, Reilmann R, Hayden MR, Tabrizi SJ, Mackay AL, Leavitt BR. Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease. Neurology 75(19):1702-10. (2010) PMID 21060093 40 Xie Y, Hayden MR, Xu B. BDNF overexpression in the forebrain rescues Huntington's disease phenotypes in YAC128 mice. J. Neurosci. 30(44):14708-18. (2010) PMID 21048129 41 Metzler M, Gan L, Mazarei G, Graham RK, Liu L, Bissada N, Lu G, Leavitt BR, Hayden MR. Phosphorylation of huntingtin at Ser421 in YAC128 neurons is associated with protection of YAC128 neurons from NMDA-mediated excitotoxicity and is modulated by PP1 and PP2A. J. Neurosci. 30(43):14318-29. (2010) PMID 20980587 42 Simpson JM, Gil-Mohapel J, Pouladi MA, Ghilan M, Xie Y, Hayden MR, Christie BR. Altered adult hippocampal neurogenesis in the YAC128 transgenic mouse model of Huntington disease. Neurobiol. Dis. 41(2):249-60. (2010) PMID 20875859 43 Loo TT, Ross CJ, Sistonen J, Visscher H, Madadi P, Koren G, Hayden MR, Carleton BC. Pharmacogenomics and active surveillance for serious adverse drug reactions in children. Pharmacogenomics 11(9):1269-85. (2010) PMID 20860467 44 Madadi P, Hildebrandt D, Gong IY, Schwarz UI, Ciszkowski C, Ross CJ, Sistonen J, Carleton BC, Hayden MR, Lauwers AE, Koren G. Fatal hydrocodone overdose in a child: pharmacogenetics and drug interactions. Pediatrics 126(4):e986-9. (2010) PMID 20837591 45 Madadi P, Joly Y, Avard D, Chitayat DC, Smith MA, Ross CJ, Carleton BC, Hayden MR, Koren G. Communicating pharmacogenetic research results to breastfeeding mothers taking codeine: a pilot study of perceptions and benefits. Clin. Pharmacol. Ther. 88(6):792-5. (2010) PMID 20739920 46 Valenza M, Leoni V, Karasinska JM, Petricca L, Fan J, Carroll J, Pouladi MA, Fossale E, Nguyen HP, Riess O, MacDonald M, Wellington C, DiDonato S, Hayden M, Cattaneo E. Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes. J. Neurosci. 30(32):10844-50. (2010) PMID 20702713 47 Huang K, Kang MH, Askew C, Kang R, Sanders SS, Wan J, Davis NG, Hayden MR. Palmitoylation and function of glial glutamate transporter-1 is reduced in the YAC128 mouse model of Huntington disease. Neurobiol. Dis. 40(1):207-15. (2010) PMID 20685337 48 Kang MH, Singaraja R, Hayden MR. Adenosine-triphosphate-binding cassette transporter-1 trafficking and function. Trends Cardiovasc. Med. 20(2):41-9. (2010) PMID 20656214 49 Ross CJ, Visscher H, Sistonen J, Brunham LR, Pussegoda K, Loo TT, Rieder MJ, Koren G, Carleton BC, Hayden MR, . The Canadian Pharmacogenomics Network for Drug Safety: a model for safety pharmacology. Thyroid 20(7):681-7. (2010) PMID 20578893 50 Williams JK, Erwin C, Juhl AR, Mengeling M, Bombard Y, Hayden MR, Quaid K, Shoulson I, Taylor S, Paulsen JS, . In their own words: reports of stigma and genetic discrimination by people at risk for Huntington disease in the International RESPOND-HD study. Am. J. Med. Genet. B Neuropsychiatr. Genet. 153B(6):1150-9. (2010) PMID 20468062 51 Erwin C, Williams JK, Juhl AR, Mengeling M, Mills JA, Bombard Y, Hayden MR, Quaid K, Shoulson I, Taylor S, Paulsen JS, . Perception, experience, and response to genetic discrimination in Huntington disease: the international RESPOND-HD study. Am. J. Med. Genet. B Neuropsychiatr. Genet. 153B(5):1081-93. (2010) PMID 20468061 52 Kruit JK, Brunham LR, Verchere CB, Hayden MR. HDL and LDL cholesterol significantly influence beta-cell function in type 2 diabetes mellitus. Curr. Opin. Lipidol. 21(3):178-85. (2010) PMID 20463468 53 Brunham LR, Kruit JK, Hayden MR, Verchere CB. Cholesterol in beta-cell dysfunction: the emerging connection between HDL cholesterol and type 2 diabetes. Curr. Diab. Rep. 10(1):55-60. (2010) PMID 20425068 54 Acuña-Alonzo V, Flores-Dorantes T, Kruit JK, Villarreal-Molina T, Arellano-Campos O, Hünemeier T, Moreno-Estrada A, Ortiz-López MG, Villamil-Ramírez H, León-Mimila P, Villalobos-Comparan M, Jacobo-Albavera L, Ramírez-Jiménez S, Sikora M, Zhang LH, Pape TD, Granados-Silvestre Mde A, Montufar-Robles I, Tito-Alvarez AM, Zurita-Salinas C, Bustos-Arriaga J, Cedillo-Barrón L, Gómez-Trejo C, Barquera-Lozano R, Vieira-Filho JP, Granados J, Romero-Hidalgo S, Huertas-Vázquez A, González-Martín A, Gorostiza A, Bonatto SL, Rodríguez-Cruz M, Wang L, Tusié-Luna T, Aguilar-Salinas CA, Lisker R, Moises RS, Menjivar M, Salzano FM, Knowler WC, Bortolini MC, Hayden MR, Baier LJ, Canizales-Quinteros S. A functional ABCA1 gene variant is associated with low HDL-cholesterol levels and shows evidence of positive selection in Native Americans. Hum. Mol. Genet. 19(14):2877-85. (2010) PMID 20418488 55 Ding YL, Wang YH, Huang W, Liu G, Ross C, Hayden MR, Yang JK. Glucose intolerance and decreased early insulin response in mice with severe hypertriglyceridemia. Exp. Biol. Med. (Maywood) 235(1):40-6. (2010) PMID 20404017 56 Kruit JK, Kremer PH, Dai L, Tang R, Ruddle P, de Haan W, Brunham LR, Verchere CB, Hayden MR. Cholesterol efflux via ATP-binding cassette transporter A1 (ABCA1) and cholesterol uptake via the LDL receptor influences cholesterol-induced impairment of beta cell function in mice. Diabetologia 53(6):1110-9. (2010) PMID 20229095 57 van Leuven SI, van Wijk DF, Volger OL, de Vries JP, van der Loos CM, de Kleijn DV, Horrevoets AJ, Tak PP, van der Wal AC, de Boer OJ, Pasterkamp G, Hayden MR, Kastelein JJ, Stroes ES. Mycophenolate mofetil attenuates plaque inflammation in patients with symptomatic carotid artery stenosis. Atherosclerosis 211(1):231-6. (2010) PMID 20202636 58 Chung S, Timmins JM, Duong M, Degirolamo C, Rong S, Sawyer JK, Singaraja RR, Hayden MR, Maeda N, Rudel LL, Shelness GS, Parks JS. Targeted deletion of hepatocyte ABCA1 leads to very low density lipoprotein triglyceride overproduction and low density lipoprotein hypercatabolism. J. Biol. Chem. 285(16):12197-209. (2010) PMID 20178985 59 Milnerwood AJ, Gladding CM, Pouladi MA, Kaufman AM, Hines RM, Boyd JD, Ko RW, Vasuta OC, Graham RK, Hayden MR, Murphy TH, Raymond LA. Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice. Neuron 65(2):178-90. (2010) PMID 20152125 60 Pouladi MA, Xie Y, Skotte NH, Ehrnhoefer DE, Graham RK, Kim JE, Bissada N, Yang XW, Paganetti P, Friedlander RM, Leavitt BR, Hayden MR. Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression. Hum. Mol. Genet. 19(8):1528-38. (2010) PMID 20097678 61 Becanovic K, Pouladi MA, Lim RS, Kuhn A, Pavlidis P, Luthi-Carter R, Hayden MR, Leavitt BR. Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis. Hum. Mol. Genet. 19(8):1438-52. (2010) PMID 20089533 62 Vergeer M, Brunham LR, Koetsveld J, Kruit JK, Verchere CB, Kastelein JJ, Hayden MR, Stroes ES. Carriers of loss-of-function mutations in ABCA1 display pancreatic beta-cell dysfunction. Diabetes Care 33(4):869-74. (2010) PMID 20067955 63 Wiens MO, Son WK, Ross C, Hayden M, Carleton B. Cases: Cocaine adulterant linked to neutropenia. CMAJ 182(1):57-9. (2010) PMID 19969562 64 Glier MB, Pissios P, Babich SL, Macdonald ML, Hayden MR, Maratos-Flier E, Gibson WT. The metabolic phenotype of SCD1-deficient mice is independent of melanin-concentrating hormone. Peptides 31(1):123-9. (2010) PMID 19883709 65 Olivecrona G, Ehrenborg E, Semb H, Makoveichuk E, Lindberg A, Hayden MR, Gin P, Davies BS, Weinstein MM, Fong LG, Beigneux AP, Young SG, Olivecrona T, Hernell O. Mutation of conserved cysteines in the Ly6 domain of GPIHBP1 in familial chylomicronemia. 51(6):1535-45. (2009) PMID 20026666 66 Thompson LM, Aiken CT, Kaltenbach LS, Agrawal N, Illes K, Khoshnan A, Martinez-Vincente M, Arrasate M, O'Rourke JG, Khashwji H, Lukacsovich T, Zhu YZ, Lau AL, Massey A, Hayden MR, Zeitlin SO, Finkbeiner S, Green KN, LaFerla FM, Bates G, Huang L, Patterson PH, Lo DC, Cuervo AM, Marsh JL, Steffan JS. IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. J. Cell Biol. 187(7):1083-99. (2009) PMID 20026656 67 Okamoto S, Pouladi MA, Talantova M, Yao D, Xia P, Ehrnhoefer DE, Zaidi R, Clemente A, Kaul M, Graham RK, Zhang D, Vincent Chen HS, Tong G, Hayden MR, Lipton SA. Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat. Med. 15(12):1407-13. (2009) PMID 19915593 68 Ross CJ, Katzov-Eckert H, Dubé MP, Brooks B, Rassekh SR, Barhdadi A, Feroz-Zada Y, Visscher H, Brown AM, Rieder MJ, Rogers PC, Phillips MS, Carleton BC, Hayden MR, . Genetic variants in TPMT and COMT are associated with hearing loss in children receiving cisplatin chemotherapy. Nat. Genet. 42(12):1345-9. (2009) PMID 19898482 69 Fan J, Cowan CM, Zhang LY, Hayden MR, Raymond LA. Interaction of postsynaptic density protein-95 with NMDA receptors influences excitotoxicity in the yeast artificial chromosome mouse model of Huntington's disease. J. Neurosci. 29(35):10928-38. (2009) PMID 19726651 70 Macdonald ML, Bissada N, Vallance BA, Hayden MR. Absence of stearoyl-CoA desaturase-1 does not promote DSS-induced acute colitis. Biochim. Biophys. Acta 1791(12):1166-72. (2009) PMID 19695343 71 Visscher H, Ross CJ, Dubé MP, Brown AM, Phillips MS, Carleton BC, Hayden MR. Application of principal component analysis to pharmacogenomic studies in Canada. Pharmacogenomics J. 9(6):362-72. (2009) PMID 19652663 72 Singaraja RR, Kang MH, Vaid K, Sanders SS, Vilas GL, Arstikaitis P, Coutinho J, Drisdel RC, El-Husseini Ael D, Green WN, Berthiaume L, Hayden MR. Palmitoylation of ATP-binding cassette transporter A1 is essential for its trafficking and function. Circ. Res. 105(2):138-47. (2009) PMID 19556522 73 Langbehn DR, Hayden MR, Paulsen JS, . CAG-repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches. Am. J. Med. Genet. B Neuropsychiatr. Genet. (2009) PMID 19548255 74 Bombard Y, Veenstra G, Friedman JM, Creighton S, Currie L, Paulsen JS, Bottorff JL, Hayden MR, . Perceptions of genetic discrimination among people at risk for Huntington's disease: a cross sectional survey. BMJ 338:b2175. (2009) PMID 19509425 75 Carleton B, Poole R, Smith M, Leeder J, Ghannadan R, Ross C, Phillips M, Hayden M. Adverse drug reaction active surveillance: developing a national network in Canada's children's hospitals. 18(8):713-21. (2009) PMID 19507171 76 Semaka A, Collins JA, Hayden MR. Unstable familial transmissions of Huntington disease alleles with 27-35 CAG repeats (intermediate alleles). Am. J. Med. Genet. B Neuropsychiatr. Genet. 153B(1):314-20. (2009) PMID 19455596 77 Wong E, Carleton BC, Wright DF, Smith MA, Verbeek L, Hildebrand CA, Stannard P, Vaillancourt R, Elliot-Miller P, Ross CJ, Hayden MR. Genotypic Approaches to Therapy in Children (GATC): using information technology to improve drug safety. Stud Health Technol Inform 143(1):209-14. (2009) PMID 19380938 78 Xian X, Liu T, Yu J, Wang Y, Miao Y, Zhang J, Yu Y, Ross C, Karasinska JM, Hayden MR, Liu G, Chui D. Presynaptic defects underlying impaired learning and memory function in lipoprotein lipase-deficient mice. J. Neurosci. 29(14):4681-5. (2009) PMID 19357293 79 Huang K, Sanders S, Singaraja R, Orban P, Cijsouw T, Arstikaitis P, Yanai A, Hayden MR, El-Husseini A. Neuronal palmitoyl acyl transferases exhibit distinct substrate specificity. FASEB J. 23(8):2605-15. (2009) PMID 19299482 80 Karasinska JM, Rinninger F, Lütjohann D, Ruddle P, Franciosi S, Kruit JK, Singaraja RR, Hirsch-Reinshagen V, Fan J, Brunham LR, Bissada N, Ramakrishnan R, Wellington CL, Parks JS, Hayden MR. Specific loss of brain ABCA1 increases brain cholesterol uptake and influences neuronal structure and function. J. Neurosci. 29(11):3579-89. (2009) PMID 19295162 81 Warby SC, Montpetit A, Hayden AR, Carroll JB, Butland SL, Visscher H, Collins JA, Semaka A, Hudson TJ, Hayden MR. CAG expansion in the Huntington disease gene is associated with a specific and targetable predisposing haplogroup. Am. J. Hum. Genet. 84(3):351-66. (2009) PMID 19249009 82 Joshi PR, Wu NP, André VM, Cummings DM, Cepeda C, Joyce JA, Carroll JB, Leavitt BR, Hayden MR, Levine MS, Bamford NS. Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease. J. Neurosci. 29(8):2414-27. (2009) PMID 19244517 83 Graham RK, Pouladi MA, Joshi P, Lu G, Deng Y, Wu NP, Figueroa BE, Metzler M, André VM, Slow EJ, Raymond L, Friedlander R, Levine MS, Leavitt BR, Hayden MR. Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease. J. Neurosci. 29(7):2193-204. (2009) PMID 19228972 84 Pouladi MA, Graham RK, Karasinska JM, Xie Y, Santos RD, Petersén A, Hayden MR. Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin. Brain 132(Pt 4):919-32. (2009) PMID 19224899 85 Ratovitski T, Gucek M, Jiang H, Chighladze E, Waldron E, D'Ambola J, Hou Z, Liang Y, Poirier MA, Hirschhorn RR, Graham R, Hayden MR, Cole RN, Ross CA. Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells. J. Biol. Chem. 284(16):10855-67. (2009) PMID 19204007 86 Brunham LR, Singaraja RR, Duong M, Timmins JM, Fievet C, Bissada N, Kang MH, Samra A, Fruchart JC, McManus B, Staels B, Parks JS, Hayden MR. Tissue-specific roles of ABCA1 influence susceptibility to atherosclerosis. Arterioscler. Thromb. Vasc. Biol. 29(4):548-54. (2009) PMID 19201688 87 Vaessen SF, Dallinga-Thie GM, Ross CJ, Splint LJ, Castellani LW, Rensen PC, Hayden MR, Schaap FG, Kuivenhoven JA. Plasma apolipoprotein AV levels in mice are positively associated with plasma triglyceride levels. J. Lipid Res. 50(5):880-4. (2009) PMID 19141870 88 MacDonald ML, van Eck M, Hildebrand RB, Wong BW, Bissada N, Ruddle P, Kontush A, Hussein H, Pouladi MA, Chapman MJ, Fievet C, van Berkel TJ, Staels B, McManus BM, Hayden MR. Despite antiatherogenic metabolic characteristics, SCD1-deficient mice have increased inflammation and atherosclerosis. Arterioscler. Thromb. Vasc. Biol. 29(3):341-7. (2009) PMID 19095997 89 Bartha I, Dinya T, Seres I, Paragh G, Ross C, Hayden MR, Biró S, Vargha G. Acute hypertriglyceridemic pancreatitis during pregnancy due to homozygous lipoprotein lipase gene mutation. Clin. Chim. Acta 400(1-2):137-8. (2009) PMID 19000906 90 Yang F, Wang Y, Sternfeld L, Rodriguez JA, Ross C, Hayden MR, Carriere F, Liu G, Schulz I. The role of free fatty acids, pancreatic lipase and Ca+ signalling in injury of isolated acinar cells and pancreatitis model in lipoprotein lipase-deficient mice. 195(1):13-28. (2009) PMID 18983441 91 Madadi P, Ross CJ, Hayden MR, Carleton BC, Gaedigk A, Leeder JS, Koren G. Pharmacogenetics of neonatal opioid toxicity following maternal use of codeine during breastfeeding: a case-control study. Clin. Pharmacol. Ther. 85(1):31-5. (2009) PMID 18719619 92 Cowan CM, Fan MM, Fan J, Shehadeh J, Zhang LY, Graham RK, Hayden MR, Raymond LA. Polyglutamine-modulated striatal calpain activity in YAC transgenic huntington disease mouse model: impact on NMDA receptor function and toxicity. J. Neurosci. 28(48):12725-35. (2008) PMID 19036965 93 Warby SC, Doty CN, Graham RK, Shively J, Singaraja RR, Hayden MR. Phosphorylation of huntingtin reduces the accumulation of its nuclear fragments. Mol. Cell. Neurosci. (2008) PMID 18992820 94 Brunham LR, Kastelein JJ, Hayden MR. ABCA1 gene mutations, HDL cholesterol levels, and risk of ischemic heart disease. JAMA 300(17):1997-8; author reply 1998. (2008) PMID 18984885 95 Wang Y, Sternfeld L, Yang F, Rodriguez JA, Ross C, Hayden MR, Carriere F, Liu G, Hofer W, Schulz I. Enhanced susceptibility to pancreatitis in severe hypertriglyceridemic lipoprotein lipase deficient mice and agonist-like function of pancreatic lipase in pancreatic cells. Gut 195(1):13-28. (2008) PMID 18936103 96 Stroes ES, Nierman MC, Meulenberg JJ, Franssen R, Twisk J, Henny CP, Maas MM, Zwinderman AH, Ross C, Aronica E, High KA, Levi MM, Hayden MR, Kastelein JJ, Kuivenhoven JA. Intramuscular administration of AAV1-lipoprotein lipase S447X lowers triglycerides in lipoprotein lipase-deficient patients. Arterioscler. Thromb. Vasc. Biol. 28(12):2303-4. (2008) PMID 18802015 97 Björkqvist M, Wild EJ, Thiele J, Silvestroni A, Andre R, Lahiri N, Raibon E, Lee RV, Benn CL, Soulet D, Magnusson A, Woodman B, Landles C, Pouladi MA, Hayden MR, Khalili-Shirazi A, Lowdell MW, Brundin P, Bates GP, Leavitt BR, Möller T, Tabrizi SJ. A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J. Exp. Med. 205(8):1869-77. (2008) PMID 18625748 98 Wang CE, Tydlacka S, Orr AL, Yang SH, Graham RK, Hayden MR, Li S, Chan AW, Li XJ. Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum. Mol. Genet. 17(17):2738-51. (2008) PMID 18558632 99 Zhang H, Li Q, Graham RK, Slow E, Hayden MR, Bezprozvanny I. Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease. Neurobiol. Dis. 31(1):80-8. (2008) PMID 18502655 100 Warby SC, Doty CN, Graham RK, Carroll JB, Yang YZ, Singaraja RR, Overall CM, Hayden MR. Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus. Hum. Mol. Genet. 17(15):2390-404. (2008) PMID 18445618 101 Lerch JP, Carroll JB, Dorr A, Spring S, Evans AC, Hayden MR, Sled JG, Henkelman RM. Cortical thickness measured from MRI in the YAC128 mouse model of Huntington's disease. Neuroimage 41(2):243-51. (2008) PMID 18387826 102 Paulsen JS, Langbehn DR, Stout JC, Aylward E, Ross CA, Nance M, Guttman M, Johnson S, MacDonald M, Beglinger LJ, Duff K, Kayson E, Biglan K, Shoulson I, Oakes D, Hayden M, . Detection of Huntington's disease decades before diagnosis: the Predict-HD study. J. Neurol. Neurosurg. Psychiatr. 79(8):874-80. (2008) PMID 18096682 103 Zhang X, Qi R, Xian X, Yang F, Blackstein M, Deng X, Fan J, Ross C, Karasinska J, Hayden MR, Liu G. Spontaneous atherosclerosis in aged lipoprotein lipase-deficient mice with severe hypertriglyceridemia on a normal chow diet. Circ. Res. 102(2):250-6. (2008) PMID 18032735 104 Fan MM, Zhang H, Hayden MR, Pelech SL, Raymond LA. Protective up-regulation of CK2 by mutant huntingtin in cells co-expressing NMDA receptors. J. Neurochem. 104(3):790-805. (2008) PMID 17971125 105 MacDonald ML, Singaraja RR, Bissada N, Ruddle P, Watts R, Karasinska JM, Gibson WT, Fievet C, Vance JE, Staels B, Hayden MR. Absence of stearoyl-CoA desaturase-1 ameliorates features of the metabolic syndrome in LDLR-deficient mice. J. Lipid Res. 49(1):217-29. (2008) PMID 17960025 106 Bombard Y, Penziner E, Suchowersky O, Guttman M, Paulsen JS, Bottorff JL, Hayden MR. Engagement with genetic discrimination: concerns and experiences in the context of Huntington disease. Eur. J. Hum. Genet. 16(3):279-89. (2008) PMID 17957229 107 Penziner E, Williams JK, Erwin C, Bombard Y, Wallis A, Beglinger LJ, Hayden MR, Paulsen JS. Perceptions of discrimination among persons who have undergone predictive testing for Huntington's disease. Am. J. Med. Genet. B Neuropsychiatr. Genet. 147(3):320-5. (2008) PMID 17948904 108 Lerch JP, Carroll JB, Spring S, Bertram LN, Schwab C, Hayden MR, Henkelman RM. Automated deformation analysis in the YAC128 Huntington disease mouse model. Neuroimage 39(1):32-9. (2008) PMID 17942324 109 Ratovitski T, Nakamura M, D'Ambola J, Chighladze E, Liang Y, Wang W, Graham R, Hayden MR, Borchelt DR, Hirschhorn RR, Ross CA. N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease. Cell Cycle 6(23):2970-81. (2007) PMID 18156806 110 Fernandes HB, Baimbridge KG, Church J, Hayden MR, Raymond LA. Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease. J. Neurosci. 27(50):13614-23. (2007) PMID 18077673 111 Parker JA, Metzler M, Georgiou J, Mage M, Roder JC, Rose AM, Hayden MR, Néri C. Huntingtin-interacting protein 1 influences worm and mouse presynaptic function and protects Caenorhabditis elegans neurons against mutant polyglutamine toxicity. J. Neurosci. 27(41):11056-64. (2007) PMID 17928447 112 Gibson WT, Hayden MR. Mycophenolate mofetil and atherosclerosis: results of animal and human studies. Ann. N. Y. Acad. Sci. 1110:209-21. (2007) PMID 17911436 113 Ross CJ, Carleton B, Warn DG, Stenton SB, Rassekh SR, Hayden MR. Genotypic approaches to therapy in children: a national active surveillance network (GATC) to study the pharmacogenomics of severe adverse drug reactions in children. Ann. N. Y. Acad. Sci. 1110:177-92. (2007) PMID 17911433 114 Valenza M, Carroll JB, Leoni V, Bertram LN, Björkhem I, Singaraja RR, Di Donato S, Lutjohann D, Hayden MR, Cattaneo E. Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation. Hum. Mol. Genet. 16(18):2187-98. (2007) PMID 17613541 115 Johnson SA, Stout JC, Solomon AC, Langbehn DR, Aylward EH, Cruce CB, Ross CA, Nance M, Kayson E, Julian-Baros E, Hayden MR, Kieburtz K, Guttman M, Oakes D, Shoulson I, Beglinger L, Duff K, Penziner E, Paulsen JS, . Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease. Brain 130(Pt 7):1732-44. (2007) PMID 17584778 116 Benn CL, Slow EJ, Farrell LA, Graham R, Deng Y, Hayden MR, Cha JH. Glutamate receptor abnormalities in the YAC128 transgenic mouse model of Huntington's disease. Neuroscience 147(2):354-72. (2007) PMID 17544587 117 Kuhn A, Goldstein DR, Hodges A, Strand AD, Sengstag T, Kooperberg C, Becanovic K, Pouladi MA, Sathasivam K, Cha JH, Hannan AJ, Hayden MR, Leavitt BR, Dunnett SB, Ferrante RJ, Albin R, Shelbourne P, Delorenzi M, Augood SJ, Faull RL, Olson JM, Bates GP, Jones L, Luthi-Carter R. Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum. Mol. Genet. 16(15):1845-61. (2007) PMID 17519223 118 Butland SL, Devon RS, Huang Y, Mead CL, Meynert AM, Neal SJ, Lee SS, Wilkinson A, Yang GS, Yuen MM, Hayden MR, Holt RA, Leavitt BR, Ouellette BF. CAG-encoded polyglutamine length polymorphism in the human genome. BMC Genomics 8:126. (2007) PMID 17519034 119 Goldberg YP, MacFarlane J, MacDonald ML, Thompson J, Dube MP, Mattice M, Fraser R, Young C, Hossain S, Pape T, Payne B, Radomski C, Donaldson G, Ives E, Cox J, Younghusband HB, Green R, Duff A, Boltshauser E, Grinspan GA, Dimon JH, Sibley BG, Andria G, Toscano E, Kerdraon J, Bowsher D, Pimstone SN, Samuels ME, Sherrington R, Hayden MR. Loss-of-function mutations in the Nav1.7 gene underlie congenital indifference to pain in multiple human populations. Clin. Genet. 71(4):311-9. (2007) PMID 17470132 120 Giacomini KM, Krauss RM, Roden DM, Eichelbaum M, Hayden MR, Nakamura Y. When good drugs go bad. Nature 446(7139):975-7. (2007) PMID 17460642 121 Van Raamsdonk JM, Murphy Z, Selva DM, Hamidizadeh R, Pearson J, Petersén A, Björkqvist M, Muir C, Mackenzie IR, Hammond GL, Vogl AW, Hayden MR, Leavitt BR. Testicular degeneration in Huntington disease. Neurobiol. Dis. 26(3):512-20. (2007) PMID 17433700 122 Ross CJ, Katzov H, Carleton B, Hayden MR. Pharmacogenomics and its implications for autoimmune disease. J. Autoimmun. 28(2-3):122-8. (2007) PMID 17418528 123 Fan MM, Fernandes HB, Zhang LY, Hayden MR, Raymond LA. Altered NMDA receptor trafficking in a yeast artificial chromosome transgenic mouse model of Huntington's disease. J. Neurosci. 27(14):3768-79. (2007) PMID 17409241 124 Van Raamsdonk JM, Warby SC, Hayden MR. Selective degeneration in YAC mouse models of Huntington disease. Brain Res. Bull. 72(2-3):124-31. (2007) PMID 17352936 125 Metzler M, Gan L, Wong TP, Liu L, Helm J, Liu L, Georgiou J, Wang Y, Bissada N, Cheng K, Roder JC, Wang YT, Hayden MR. NMDA receptor function and NMDA receptor-dependent phosphorylation of huntingtin is altered by the endocytic protein HIP1. J. Neurosci. 27(9):2298-308. (2007) PMID 17329427 126 Brunham LR, Kruit JK, Pape TD, Timmins JM, Reuwer AQ, Vasanji Z, Marsh BJ, Rodrigues B, Johnson JD, Parks JS, Verchere CB, Hayden MR. Beta-cell ABCA1 influences insulin secretion, glucose homeostasis and response to thiazolidinedione treatment. Nat. Med. 13(3):340-7. (2007) PMID 17322896 127 Bombard Y, Penziner E, Decolongon J, Klimek ML, Creighton S, Suchowersky O, Guttman M, Paulsen JS, Bottorff JL, Hayden MR. Managing genetic discrimination: strategies used by individuals found to have the Huntington disease mutation. Clin. Genet. 71(3):220-31. (2007) PMID 17309644 128 Solomon AC, Stout JC, Johnson SA, Langbehn DR, Aylward EH, Brandt J, Ross CA, Beglinger L, Hayden MR, Kieburtz K, Kayson E, Julian-Baros E, Duff K, Guttman M, Nance M, Oakes D, Shoulson I, Penziner E, Paulsen JS, . Verbal episodic memory declines prior to diagnosis in Huntington's disease. 45(8):1767-76. (2007) PMID 17303196 129 Van Raamsdonk JM, Metzler M, Slow E, Pearson J, Schwab C, Carroll J, Graham RK, Leavitt BR, Hayden MR. Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain. Neurobiol. Dis. 26(1):189-200. (2007) PMID 17276692 130 Hirsch-Reinshagen V, Chan JY, Wilkinson A, Tanaka T, Fan J, Ou G, Maia LF, Singaraja RR, Hayden MR, Wellington CL. Physiologically regulated transgenic ABCA1 does not reduce amyloid burden or amyloid-beta peptide levels in vivo. J. Lipid Res. 48(4):914-23. (2007) PMID 17235115 131 Wang J, Xian X, Huang W, Chen L, Wu L, Zhu Y, Fan J, Ross C, Hayden MR, Liu G. Expression of LPL in endothelial-intact artery results in lipid deposition and vascular cell adhesion molecule-1 upregulation in both LPL and ApoE-deficient mice. Arterioscler. Thromb. Vasc. Biol. 27(1):197-203. (2007) PMID 17038632 132 Khatchadourian K, Smith CE, Metzler M, Gregory M, Hayden MR, Cyr DG, Hermo L. Structural abnormalities in spermatids together with reduced sperm counts and motility underlie the reproductive defect in HIP1-/- mice. Mol. Reprod. Dev. 74(3):341-59. (2007) PMID 16967501 133 Van Raamsdonk JM, Pearson J, Murphy Z, Hayden MR, Leavitt BR. Wild-type huntingtin ameliorates striatal neuronal atrophy but does not prevent other abnormalities in the YAC128 mouse model of Huntington disease. 7:80. (2006) PMID 17147801 134 Zhang Y, Leavitt BR, van Raamsdonk JM, Dragatsis I, Goldowitz D, MacDonald ME, Hayden MR, Friedlander RM. Huntingtin inhibits caspase-3 activation. EMBO J. 25(24):5896-906. (2006) PMID 17124493 135 Oliveira JM, Chen S, Almeida S, Riley R, Gonçalves J, Oliveira CR, Hayden MR, Nicholls DG, Ellerby LM, Rego AC. Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors. J. Neurosci. 26(43):11174-86. (2006) PMID 17065457 136 Houde C, Dickinson RJ, Houtzager VM, Cullum R, Montpetit R, Metzler M, Simpson EM, Roy S, Hayden MR, Hoodless PA, Nicholson DW. Hippi is essential for node cilia assembly and Sonic hedgehog signaling. Dev. Biol. 300(2):523-33. (2006) PMID 17027958 137 Leavitt BR, Hayden MR. Is tetrabenazine safe and effective for suppressing chorea in Huntington's disease? 2(10):536-7. (2006) PMID 16990826 138 Semaka A, Creighton S, Warby S, Hayden MR. Predictive testing for Huntington disease: interpretation and significance of intermediate alleles. Clin. Genet. 70(4):283-94. (2006) PMID 16965319 139 Kishikawa S, Li JL, Gillis T, Hakky MM, Warby S, Hayden M, MacDonald ME, Myers RH, Gusella JF. Brain-derived neurotrophic factor does not influence age at neurologic onset of Huntington's disease. Neurobiol. Dis. 24(2):280-5. (2006) PMID 16962786 140 Brunham LR, Kruit JK, Pape TD, Parks JS, Kuipers F, Hayden MR. Tissue-specific induction of intestinal ABCA1 expression with a liver X receptor agonist raises plasma HDL cholesterol levels. Circ. Res. 99(7):672-4. (2006) PMID 16946132 141 Singaraja RR, Van Eck M, Bissada N, Zimetti F, Collins HL, Hildebrand RB, Hayden A, Brunham LR, Kang MH, Fruchart JC, Van Berkel TJ, Parks JS, Staels B, Rothblat GH, Fiévet C, Hayden MR. Both hepatic and extrahepatic ABCA1 have discrete and essential functions in the maintenance of plasma high-density lipoprotein cholesterol levels in vivo. Circulation 114(12):1301-9. (2006) PMID 16940190 142 Li JL, Hayden MR, Warby SC, Durr A, Morrison PJ, Nance M, Ross CA, Margolis RL, Rosenblatt A, Squitieri F, Frati L, Gómez-Tortosa E, García CA, Suchowersky O, Klimek ML, Trent RJ, McCusker E, Novelletto A, Frontali M, Paulsen JS, Jones R, Ashizawa T, Lazzarini A, Wheeler VC, Prakash R, Xu G, Djoussé L, Mysore JS, Gillis T, Hakky M, Cupples LA, Saint-Hilaire MH, Cha JH, Hersch SM, Penney JB, Harrison MB, Perlman SL, Zanko A, Abramson RK, Lechich AJ, Duckett A, Marder K, Conneally PM, Gusella JF, MacDonald ME, Myers RH. Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study. BMC Med. Genet. 7:71. (2006) PMID 16914060 143 Burgess BL, McIsaac SA, Naus KE, Chan JY, Tansley GH, Yang J, Miao F, Ross CJ, van Eck M, Hayden MR, van Nostrand W, St George-Hyslop P, Westaway D, Wellington CL. Elevated plasma triglyceride levels precede amyloid deposition in Alzheimer's disease mouse models with abundant A beta in plasma. Neurobiol. Dis. 24(1):114-27. (2006) PMID 16899370 144 Alvarez C, Tredwell S, De Vera M, Hayden M. The genotype-phenotype correlation of hereditary multiple exostoses. Clin. Genet. 70(2):122-30. (2006) PMID 16879194 145 Singaraja RR, Visscher H, James ER, Chroni A, Coutinho JM, Brunham LR, Kang MH, Zannis VI, Chimini G, Hayden MR. Specific mutations in ABCA1 have discrete effects on ABCA1 function and lipid phenotypes both in vivo and in vitro. Circ. Res. 99(4):389-97. (2006) PMID 16873719 146 Björkqvist M, Petersén A, Nielsen J, Ecker D, Mulder H, Hayden MR, Landwehrmeyer B, Brundin P, Leavitt BR. Cerebrospinal fluid levels of orexin-A are not a clinically useful biomarker for Huntington disease. Clin. Genet. 70(1):78-9. (2006) PMID 16813610 147 Slow EJ, Graham RK, Hayden MR. To be or not to be toxic: aggregations in Huntington and Alzheimer disease. Trends Genet. 22(8):408-11. (2006) PMID 16806565 148 Graham RK, Deng Y, Slow EJ, Haigh B, Bissada N, Lu G, Pearson J, Shehadeh J, Bertram L, Murphy Z, Warby SC, Doty CN, Roy S, Wellington CL, Leavitt BR, Raymond LA, Nicholson DW, Hayden MR. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 125(6):1179-91. (2006) PMID 16777606 149 Devon RS, Orban PC, Gerrow K, Barbieri MA, Schwab C, Cao LP, Helm JR, Bissada N, Cruz-Aguado R, Davidson TL, Witmer J, Metzler M, Lam CK, Tetzlaff W, Simpson EM, McCaffery JM, El-Husseini AE, Leavitt BR, Hayden MR. Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities. Proc. Natl. Acad. Sci. U.S.A. 103(25):9595-600. (2006) PMID 16769894 150 Paulsen JS, Hayden M, Stout JC, Langbehn DR, Aylward E, Ross CA, Guttman M, Nance M, Kieburtz K, Oakes D, Shoulson I, Kayson E, Johnson S, Penziner E, . Preparing for preventive clinical trials: the Predict-HD study. Arch. Neurol. 63(6):883-90. (2006) PMID 16769871 151 Singaraja RR, Stahmer B, Brundert M, Merkel M, Heeren J, Bissada N, Kang M, Timmins JM, Ramakrishnan R, Parks JS, Hayden MR, Rinninger F. Hepatic ATP-binding cassette transporter A1 is a key molecule in high-density lipoprotein cholesteryl ester metabolism in mice. Arterioscler. Thromb. Vasc. Biol. 26(8):1821-7. (2006) PMID 16728652 152 Ross CJ, Twisk J, Bakker AC, Miao F, Verbart D, Rip J, Godbey T, Dijkhuizen P, Hermens WT, Kastelein JJ, Kuivenhoven JA, Meulenberg JM, Hayden MR. Correction of feline lipoprotein lipase deficiency with adeno-associated virus serotype 1-mediated gene transfer of the lipoprotein lipase S447X beneficial mutation. Hum. Gene Ther. 17(5):487-99. (2006) PMID 16716106 153 Brunham LR, Singaraja RR, Hayden MR. Variations on a gene: rare and common variants in ABCA1 and their impact on HDL cholesterol levels and atherosclerosis. Annu. Rev. Nutr. 26:105-29. (2006) PMID 16704350 154 Yanai A, Huang K, Kang R, Singaraja RR, Arstikaitis P, Gan L, Orban PC, Mullard A, Cowan CM, Raymond LA, Drisdel RC, Green WN, Ravikumar B, Rubinsztein DC, El-Husseini A, Hayden MR. Palmitoylation of huntingtin by HIP14 is essential for its trafficking and function. Nat. Neurosci. 9(6):824-31. (2006) PMID 16699508 155 Guidetti P, Bates GP, Graham RK, Hayden MR, Leavitt BR, MacDonald ME, Slow EJ, Wheeler VC, Woodman B, Schwarcz R. Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice. Neurobiol. Dis. 23(1):190-7. (2006) PMID 16697652 156 Rip J, Nierman MC, Ross CJ, Jukema JW, Hayden MR, Kastelein JJ, Stroes ES, Kuivenhoven JA. Lipoprotein lipase S447X: a naturally occurring gain-of-function mutation. Arterioscler. Thromb. Vasc. Biol. 26(6):1236-45. (2006) PMID 16574898 157 Van Raamsdonk JM, Gibson WT, Pearson J, Murphy Z, Lu G, Leavitt BR, Hayden MR. Body weight is modulated by levels of full-length huntingtin. Hum. Mol. Genet. 15(9):1513-23. (2006) PMID 16571604 158 Brunham LR, Kruit JK, Iqbal J, Fievet C, Timmins JM, Pape TD, Coburn BA, Bissada N, Staels B, Groen AK, Hussain MM, Parks JS, Kuipers F, Hayden MR. Intestinal ABCA1 directly contributes to HDL biogenesis in vivo. J. Clin. Invest. 116(4):1052-62. (2006) PMID 16543947 159 Bae BI, Hara MR, Cascio MB, Wellington CL, Hayden MR, Ross CA, Ha HC, Li XJ, Snyder SH, Sawa A. Mutant huntingtin: nuclear translocation and cytotoxicity mediated by GAPDH. Proc. Natl. Acad. Sci. U.S.A. 103(9):3405-9. (2006) PMID 16492755 160 Hegele RA. Gene therapy with lipoprotein lipase variant S447X. Arterioscler. Thromb. Vasc. Biol. 26(3):e25; author reply e25-8. (2006) PMID 16484602 161 Zhao T, Guo J, Li H, Huang W, Xian X, Ross CJ, Hayden MR, Wen Z, Liu G. Hemorheological abnormalities in lipoprotein lipase deficient mice with severe hypertriglyceridemia. Biochem. Biophys. Res. Commun. 341(4):1066-71. (2006) PMID 16460682 162 Van Eck M, Singaraja RR, Ye D, Hildebrand RB, James ER, Hayden MR, Van Berkel TJ. Macrophage ATP-binding cassette transporter A1 overexpression inhibits atherosclerotic lesion progression in low-density lipoprotein receptor knockout mice. Arterioscler. Thromb. Vasc. Biol. 26(4):929-34. (2006) PMID 16456089 163 Leavitt BR, van Raamsdonk JM, Shehadeh J, Fernandes H, Murphy Z, Graham RK, Wellington CL, Raymond LA, Hayden MR. Wild-type huntingtin protects neurons from excitotoxicity. J. Neurochem. 96(4):1121-9. (2006) PMID 16417581 164 Warby S, MacDonald M, Hayden M, Butland S, Ouellette F. FASA-57 cDNA shares no homology with coding sequence of HD gene. J. Reprod. Immunol. 69(1):9-10; author reply 11. (2006) PMID 16386309 165 van Leuven SI, Kastelein JJ, Allison AC, Hayden MR, Stroes ES. Mycophenolate mofetil (MMF): firing at the atherosclerotic plaque from different angles? Cardiovasc. Res. 69(2):341-7. (2006) PMID 16289002 166 Graham RK, Slow EJ, Deng Y, Bissada N, Lu G, Pearson J, Shehadeh J, Leavitt BR, Raymond LA, Hayden MR. Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models. Neurobiol. Dis. 21(2):444-55. (2006) PMID 16230019 167 Shehadeh J, Fernandes HB, Zeron Mullins MM, Graham RK, Leavitt BR, Hayden MR, Raymond LA. Striatal neuronal apoptosis is preferentially enhanced by NMDA receptor activation in YAC transgenic mouse model of Huntington disease. Neurobiol. Dis. 21(2):392-403. (2006) PMID 16165367 168 Brunham LR, Singaraja RR, Pape TD, Kejariwal A, Thomas PD, Hayden MR. Accurate prediction of the functional significance of single nucleotide polymorphisms and mutations in the ABCA1 gene. PLoS Genet. 1(6):e83. (2005) PMID 16429166 169 Hayden MR, Bombard Y. Psychosocial effects of predictive testing for Huntington's disease. Adv Neurol 96:226-39. (2005) PMID 16383222 170 Van Raamsdonk JM, Murphy Z, Slow EJ, Leavitt BR, Hayden MR. Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 14(24):3823-35. (2005) PMID 16278236 171 Rip J, Nierman MC, Sierts JA, Petersen W, Van den Oever K, Van Raalte D, Ross CJ, Hayden MR, Bakker AC, Dijkhuizen P, Hermens WT, Twisk J, Stroes E, Kastelein JJ, Kuivenhoven JA, Meulenberg JM. Gene therapy for lipoprotein lipase deficiency: working toward clinical application. Hum. Gene Ther. 16(11):1276-86. (2005) PMID 16259561 172 Hirsch-Reinshagen V, Maia LF, Burgess BL, Blain JF, Naus KE, McIsaac SA, Parkinson PF, Chan JY, Tansley GH, Hayden MR, Poirier J, Van Nostrand W, Wellington CL. The absence of ABCA1 decreases soluble ApoE levels but does not diminish amyloid deposition in two murine models of Alzheimer disease. J. Biol. Chem. 280(52):43243-56. (2005) PMID 16207707 173 Van Raamsdonk JM, Pearson J, Bailey CD, Rogers DA, Johnson GV, Hayden MR, Leavitt BR. Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease. J. Neurochem. 95(1):210-20. (2005) PMID 16181425 174 van Leuven SI, Kastelein JJ, Hayden MR, d'Cruz D, Hughes GR, Stroes ES. Cardiovascular disease in systemic lupus erythematosus: has the time for action come? Curr. Opin. Lipidol. 16(5):501-6. (2005) PMID 16148533 175 Jenkins BG, Andreassen OA, Dedeoglu A, Leavitt B, Hayden M, Borchelt D, Ross CA, Ferrante RJ, Beal MF. Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease. J. Neurochem. 95(2):553-62. (2005) PMID 16135087 176 Van Raamsdonk JM, Pearson J, Rogers DA, Lu G, Barakauskas VE, Barr AM, Honer WG, Hayden MR, Leavitt BR. Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease. Exp. Neurol. 196(2):266-72. (2005) PMID 16129433 177 Kegel KB, Sapp E, Yoder J, Cuiffo B, Sobin L, Kim YJ, Qin ZH, Hayden MR, Aronin N, Scott DL, Isenberg G, Goldmann WH, DiFiglia M. Huntingtin associates with acidic phospholipids at the plasma membrane. J. Biol. Chem. 280(43):36464-73. (2005) PMID 16085648 178 Slow EJ, Graham RK, Osmand AP, Devon RS, Lu G, Deng Y, Pearson J, Vaid K, Bissada N, Wetzel R, Leavitt BR, Hayden MR. Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc. Natl. Acad. Sci. U.S.A. 102(32):11402-7. (2005) PMID 16076956 179 Puri BK, Leavitt BR, Hayden MR, Ross CA, Rosenblatt A, Greenamyre JT, Hersch S, Vaddadi KS, Sword A, Horrobin DF, Manku M, Murck H. Ethyl-EPA in Huntington disease: a double-blind, randomized, placebo-controlled trial. Neurology 65(2):286-92. (2005) PMID 16043801 180 Singaraja RR, James ER, Crim J, Visscher H, Chatterjee A, Hayden MR. Alternate transcripts expressed in response to diet reflect tissue-specific regulation of ABCA1. J. Lipid Res. 46(10):2061-71. (2005) PMID 16024915 181 Ross CJ, Liu G, Kuivenhoven JA, Twisk J, Rip J, van Dop W, Excoffon KJ, Lewis SM, Kastelein JJ, Hayden MR. Complete rescue of lipoprotein lipase-deficient mice by somatic gene transfer of the naturally occurring LPLS447X beneficial mutation. Arterioscler. Thromb. Vasc. Biol. 25(10):2143-50. (2005) PMID 16002740 182 Pinto JT, Van Raamsdonk JM, Leavitt BR, Hayden MR, Jeitner TM, Thaler HT, Krasnikov BF, Cooper AJ. Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease. J. Neurochem. 94(4):1087-101. (2005) PMID 15992377 183 Van Raamsdonk JM, Pearson J, Slow EJ, Hossain SM, Leavitt BR, Hayden MR. Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J. Neurosci. 25(16):4169-80. (2005) PMID 15843620 184 Warby SC, Chan EY, Metzler M, Gan L, Singaraja RR, Crocker SF, Robertson HA, Hayden MR. Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo. Hum. Mol. Genet. 14(11):1569-77. (2005) PMID 15843398 185 Timmins JM, Lee JY, Boudyguina E, Kluckman KD, Brunham LR, Mulya A, Gebre AK, Coutinho JM, Colvin PL, Smith TL, Hayden MR, Maeda N, Parks JS. Targeted inactivation of hepatic Abca1 causes profound hypoalphalipoproteinemia and kidney hypercatabolism of apoA-I. J. Clin. Invest. 115(5):1333-42. (2005) PMID 15841208 186 Van Raamsdonk JM, Pearson J, Rogers DA, Bissada N, Vogl AW, Hayden MR, Leavitt BR. Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 14(10):1379-92. (2005) PMID 15829505 187 Coutinho JM, Singaraja RR, Kang M, Arenillas DJ, Bertram LN, Bissada N, Staels B, Fruchart JC, Fievet C, Joseph-George AM, Wasserman WW, Hayden MR. Complete functional rescue of the ABCA1-/- mouse by human BAC transgenesis. J. Lipid Res. 46(6):1113-23. (2005) PMID 15772424 188 Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinás R, Kristal BS, Hayden MR, Bezprozvanny I. Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc. Natl. Acad. Sci. U.S.A. 102(7):2602-7. (2005) PMID 15695335 189 Devon RS, Schwab C, Topp JD, Orban PC, Yang YZ, Pape TD, Helm JR, Davidson TL, Rogers DA, Gros-Louis F, Rouleau G, Horazdovsky BF, Leavitt BR, Hayden MR. Cross-species characterization of the ALS2 gene and analysis of its pattern of expression in development and adulthood. Neurobiol. Dis. 18(2):243-57. (2005) PMID 15686953 190 Legendre-Guillemin V, Metzler M, Lemaire JF, Philie J, Gan L, Hayden MR, McPherson PS. Huntingtin interacting protein 1 (HIP1) regulates clathrin assembly through direct binding to the regulatory region of the clathrin light chain. J. Biol. Chem. 280(7):6101-8. (2005) PMID 15533941 191 Van Raamsdonk JM, Hayden MR, Leavitt BR. Experimental models of Huntington disease Drug Discovery Today Review 2(4):291-297. (2005) 192 Huang K, Yanai A, Kang R, Arstikaitis P, Singaraja RR, Metzler M, Mullard A, Haigh B, Gauthier-Campbell C, Gutekunst CA, Hayden MR, El-Husseini A. Huntingtin-interacting protein HIP14 is a palmitoyl transferase involved in palmitoylation and trafficking of multiple neuronal proteins. Neuron 44(6):977-86. (2004) PMID 15603740 193 Margolis RL, Holmes SE, Rosenblatt A, Gourley L, O'Hearn E, Ross CA, Seltzer WK, Walker RH, Ashizawa T, Rasmussen A, Hayden M, Almqvist EW, Harris J, Fahn S, MacDonald ME, Mysore J, Shimohata T, Tsuji S, Potter N, Nakaso K, Adachi Y, Nakashima K, Bird T, Krause A, Greenstein P. Huntington's Disease-like 2 (HDL2) in North America and Japan. Ann. Neurol. 56(5):670-4. (2004) PMID 15468075 194 Hovingh GK, Brownlie A, Bisoendial RJ, Dube MP, Levels JH, Petersen W, Dullaart RP, Stroes ES, Zwinderman AH, de Groot E, Hayden MR, Kuivenhoven JA, Kastelein JJ. A novel apoA-I mutation (L178P) leads to endothelial dysfunction, increased arterial wall thickness, and premature coronary artery disease. J. Am. Coll. Cardiol. 44(7):1429-35. (2004) PMID 15464323 195 Guan JZ, Tamasawa N, Brunham LR, Matsui J, Murakami H, Suda T, Ochiai S, Tsutsui M, Kudou K, Satoh K, Hayden MR. A case of Tangier disease with a novel mutation in the C-terminal region of ATP-binding cassette transporter A1. Am. J. Med. Genet. A 130A(4):398-401. (2004) PMID 15384103 196 Tang TS, Tu H, Orban PC, Chan EY, Hayden MR, Bezprozvanny I. HAP1 facilitates effects of mutant huntingtin on inositol 1,4,5-trisphosphate-induced Ca release in primary culture of striatal medium spiny neurons. Eur. J. Neurosci. 20(7):1779-87. (2004) PMID 15379999 197 Ross CJ, Twisk J, Meulenberg JM, Liu G, van den Oever K, Moraal E, Hermens WT, Rip J, Kastelein JJ, Kuivenhoven JA, Hayden MR. Long-term correction of murine lipoprotein lipase deficiency with AAV1-mediated gene transfer of the naturally occurring LPL(S447X) beneficial mutation. Hum. Gene Ther. 15(9):906-19. (2004) PMID 15353045 198 Trushina E, Dyer RB, Badger JD, Ure D, Eide L, Tran DD, Vrieze BT, Legendre-Guillemin V, McPherson PS, Mandavilli BS, Van Houten B, Zeitlin S, McNiven M, Aebersold R, Hayden M, Parisi JE, Seeberg E, Dragatsis I, Doyle K, Bender A, Chacko C, McMurray CT. Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Mol. Cell. Biol. 24(18):8195-209. (2004) PMID 15340079 199 Bezprozvanny I, Hayden MR. Deranged neuronal calcium signaling and Huntington disease. Biochem. Biophys. Res. Commun. 322(4):1310-7. (2004) PMID 15336977 200 Hirsch-Reinshagen V, Zhou S, Burgess BL, Bernier L, McIsaac SA, Chan JY, Tansley GH, Cohn JS, Hayden MR, Wellington CL. Deficiency of ABCA1 impairs apolipoprotein E metabolism in brain. J. Biol. Chem. 279(39):41197-207. (2004) PMID 15269218 201 Li L, Murphy TH, Hayden MR, Raymond LA. Enhanced striatal NR2B-containing N-methyl-D-aspartate receptor-mediated synaptic currents in a mouse model of Huntington disease. J. Neurophysiol. 92(5):2738-46. (2004) PMID 15240759 202 Saleh M, Vaillancourt JP, Graham RK, Huyck M, Srinivasula SM, Alnemri ES, Steinberg MH, Nolan V, Baldwin CT, Hotchkiss RS, Buchman TG, Zehnbauer BA, Hayden MR, Farrer LA, Roy S, Nicholson DW. Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms. Nature 429(6987):75-9. (2004) PMID 15129283 203 Delatycki MB, Allen KJ, Gow P, MacFarlane J, Radomski C, Thompson J, Hayden MR, Goldberg YP, Samuels ME. A homozygous HAMP mutation in a multiply consanguineous family with pseudo-dominant juvenile hemochromatosis. Clin. Genet. 65(5):378-83. (2004) PMID 15099344 204 Lafreniere RG, MacDonald ML, Dube MP, MacFarlane J, O'Driscoll M, Brais B, Meilleur S, Brinkman RR, Dadivas O, Pape T, Platon C, Radomski C, Risler J, Thompson J, Guerra-Escobio AM, Davar G, Breakefield XO, Pimstone SN, Green R, Pryse-Phillips W, Goldberg YP, Younghusband HB, Hayden MR, Sherrington R, Rouleau GA, Samuels ME, . Identification of a novel gene (HSN2) causing hereditary sensory and autonomic neuropathy type II through the Study of Canadian Genetic Isolates. Am. J. Hum. Genet. 74(5):1064-73. (2004) PMID 15060842 205 Harper PS, Gevers S, de Wert G, Creighton S, Bombard Y, Hayden MR. Genetic testing and Huntington's disease: issues of employment. Lancet Neurol 3(4):249-52. (2004) PMID 15039038 206 Zeron MM, Fernandes HB, Krebs C, Shehadeh J, Wellington CL, Leavitt BR, Baimbridge KG, Hayden MR, Raymond LA. Potentiation of NMDA receptor-mediated excitotoxicity linked with intrinsic apoptotic pathway in YAC transgenic mouse model of Huntington's disease. Mol. Cell. Neurosci. 25(3):469-79. (2004) PMID 15033175 207 Djoussé L, Knowlton B, Hayden MR, Almqvist EW, Brinkman RR, Ross CA, Margolis RL, Rosenblatt A, Durr A, Dode C, Morrison PJ, Novelletto A, Frontali M, Trent RJ, McCusker E, Gómez-Tortosa E, Mayo Cabrero D, Jones R, Zanko A, Nance M, Abramson RK, Suchowersky O, Paulsen JS, Harrison MB, Yang Q, Cupples LA, Mysore J, Gusella JF, MacDonald ME, Myers RH. Evidence for a modifier of onset age in Huntington disease linked to the HD gene in 4p16. Neurogenetics 5(2):109-14. (2004) PMID 15029481 208 Selva DM, Hirsch-Reinshagen V, Burgess B, Zhou S, Chan J, McIsaac S, Hayden MR, Hammond GL, Vogl AW, Wellington CL. The ATP-binding cassette transporter 1 mediates lipid efflux from Sertoli cells and influences male fertility. J. Lipid Res. 45(6):1040-50. (2004) PMID 15026428 209 Langbehn DR, Brinkman RR, Falush D, Paulsen JS, Hayden MR, . A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length. Clin. Genet. 65(4):267-77. (2004) PMID 15025718 210 Gafni J, Hermel E, Young JE, Wellington CL, Hayden MR, Ellerby LM. Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus. J. Biol. Chem. 279(19):20211-20. (2004) PMID 14981075 211 Hovingh GK, Kuivenhoven JA, Bisoendial RJ, Groen AK, van Dam M, van Tol A, Wellington C, Hayden MR, Smelt AH, Kastelein JJ. HDL deficiency and atherosclerosis: lessons from Tangier disease. J. Intern. Med. 255(2):299-301. (2004) PMID 14746569 212 Qin ZH, Wang Y, Sapp E, Cuiffo B, Wanker E, Hayden MR, Kegel KB, Aronin N, DiFiglia M. Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction. J. Neurosci. 24(1):269-81. (2004) PMID 14715959 213 Hermel E, Gafni J, Propp SS, Leavitt BR, Wellington CL, Young JE, Hackam AS, Logvinova AV, Peel AL, Chen SF, Hook V, Singaraja R, Krajewski S, Goldsmith PC, Ellerby HM, Hayden MR, Bredesen DE, Ellerby LM. Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death Differ. 11(4):424-38. (2004) PMID 14713958 214 Zala D, Bensadoun JC, Pereira de Almeida L, Leavitt BR, Gutekunst CA, Aebischer P, Hayden MR, Déglon N. Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the striatum of Huntington's disease transgenic mice. Exp. Neurol. 185(1):26-35. (2004) PMID 14697316 215 Papanikolaou G, Samuels ME, Ludwig EH, MacDonald ML, Franchini PL, Dubé MP, Andres L, MacFarlane J, Sakellaropoulos N, Politou M, Nemeth E, Thompson J, Risler JK, Zaborowska C, Babakaiff R, Radomski CC, Pape TD, Davidas O, Christakis J, Brissot P, Lockitch G, Ganz T, Hayden MR, Goldberg YP. Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat. Genet. 36(1):77-82. (2004) PMID 14647275 216 Squitieri F, Almqvist EW, Cannella M, Cislaghi G, Hayden MR. Predictive testing for persons at risk for homozygosity for CAG expansion in the Huntington disease gene. Clin. Genet. 64(6):524-5. (2003) PMID 14986835 217 Hand CK, Devon RS, Gros-Louis F, Rochefort D, Khoris J, Meininger V, Bouchard JP, Camu W, Hayden MR, Rouleau GA. Mutation screening of the ALS2 gene in sporadic and familial amyotrophic lateral sclerosis. Arch. Neurol. 60(12):1768-71. (2003) PMID 14676054 218 Huntington Study Group. Dosage effects of riluzole in Huntington's disease: a multicenter placebo-controlled study. Neurology 61(11):1551-6. (2003) PMID 14663041 219 Kuivenhoven JA, Hovingh GK, van Tol A, Jauhiainen M, Ehnholm C, Fruchart JC, Brinton EA, Otvos JD, Smelt AH, Brownlee A, Zwinderman AH, Hayden MR, Kastelein JJ. Heterozygosity for ABCA1 gene mutations: effects on enzymes, apolipoproteins and lipoprotein particle size. Atherosclerosis 171(2):311-9. (2003) PMID 14644402 220 Li L, Fan M, Icton CD, Chen N, Leavitt BR, Hayden MR, Murphy TH, Raymond LA. Role of NR2B-type NMDA receptors in selective neurodegeneration in Huntington disease. Neurobiol. Aging 24(8):1113-21. (2003) PMID 14643383 221 Aylward EH, Rosenblatt A, Field K, Yallapragada V, Kieburtz K, McDermott M, Raymond LA, Almqvist EW, Hayden M, Ross CA. Caudate volume as an outcome measure in clinical trials for Huntington's disease: a pilot study. Brain Res. Bull. 62(2):137-41. (2003) PMID 14638387 222 Brunham LR, Hayden MR. Clarity is essential when using nucleotide number systems. Atherosclerosis 170(2):349. (2003) PMID 14612218 223 Almqvist EW, Brinkman RR, Wiggins S, Hayden MR, . Psychological consequences and predictors of adverse events in the first 5 years after predictive testing for Huntington's disease. Clin. Genet. 64(4):300-9. (2003) PMID 12974735 224 Zhang Y, Li M, Drozda M, Chen M, Ren S, Mejia Sanchez RO, Leavitt BR, Cattaneo E, Ferrante RJ, Hayden MR, Friedlander RM. Depletion of wild-type huntingtin in mouse models of neurologic diseases. J. Neurochem. 87(1):101-6. (2003) PMID 12969257 225 Hoogendijk CF, Scholtz CL, Pimstone SM, Ehrenborg E, Kastelein JJ, Defesche JC, Thiart R, du Plessis L, de Villiers JN, Zaahl MG, Delport R, Rubinsztein DC, Raffel LJ, Grim CE, Mediene-Benchekor S, Amouyel P, Brousseau T, Steyn K, Lombard CJ, Hayden MR, Kotze MJ. Allelic variation in the promoter region of the LDL receptor gene: analysis of an African-specific variant in the FP2 cis-acting regulatory element. Mol. Cell. Probes 17(4):175-81. (2003) PMID 12944120 226 Devon RS, Helm JR, Rouleau GA, Leitner Y, Lerman-Sagie T, Lev D, Hayden MR. The first nonsense mutation in alsin results in a homogeneous phenotype of infantile-onset ascending spastic paralysis with bulbar involvement in two siblings. Clin. Genet. 64(3):210-5. (2003) PMID 12919135 227 McCrindle BW, Ose L, Marais AD. Efficacy and safety of atorvastatin in children and adolescents with familial hypercholesterolemia or severe hyperlipidemia: a multicenter, randomized, placebo-controlled trial. J. Pediatr. 143(1):74-80. (2003) PMID 12915827 228 Li JL, Hayden MR, Almqvist EW, Brinkman RR, Durr A, Dodé C, Morrison PJ, Suchowersky O, Ross CA, Margolis RL, Rosenblatt A, Gómez-Tortosa E, Cabrero DM, Novelletto A, Frontali M, Nance M, Trent RJ, McCusker E, Jones R, Paulsen JS, Harrison M, Zanko A, Abramson RK, Russ AL, Knowlton B, Djoussé L, Mysore JS, Tariot S, Gusella MF, Wheeler VC, Atwood LD, Cupples LA, Saint-Hilaire M, Cha JH, Hersch SM, Koroshetz WJ, Gusella JF, MacDonald ME, Myers RH. A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study. Am. J. Hum. Genet. 73(3):682-7. (2003) PMID 12900792 229 Zuccato C, Tartari M, Crotti A, Goffredo D, Valenza M, Conti L, Cataudella T, Leavitt BR, Hayden MR, Timmusk T, Rigamonti D, Cattaneo E. Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat. Genet. 35(1):76-83. (2003) PMID 12881722 230 Tang TS, Tu H, Chan EY, Maximov A, Wang Z, Wellington CL, Hayden MR, Bezprozvanny I. Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron 39(2):227-39. (2003) PMID 12873381 231 Hayden MR. Predictive testing for Huntington's disease: a universal model? 2(3):141-2. (2003) PMID 12849232 232 Metzler M, Li B, Gan L, Georgiou J, Gutekunst CA, Wang Y, Torre E, Devon RS, Oh R, Legendre-Guillemin V, Rich M, Alvarez C, Gertsenstein M, McPherson PS, Nagy A, Wang YT, Roder JC, Raymond LA, Hayden MR. Disruption of the endocytic protein HIP1 results in neurological deficits and decreased AMPA receptor trafficking. EMBO J. 22(13):3254-66. (2003) PMID 12839988 233 Slow EJ, van Raamsdonk J, Rogers D, Coleman SH, Graham RK, Deng Y, Oh R, Bissada N, Hossain SM, Yang YZ, Li XJ, Simpson EM, Gutekunst CA, Leavitt BR, Hayden MR. Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 12(13):1555-67. (2003) PMID 12812983 234 Creighton S, Almqvist EW, MacGregor D, Fernandez B, Hogg H, Beis J, Welch JP, Riddell C, Lokkesmoe R, Khalifa M, MacKenzie J, Sajoo A, Farrell S, Robert F, Shugar A, Summers A, Meschino W, Allingham-Hawkins D, Chiu T, Hunter A, Allanson J, Hare H, Schween J, Collins L, Sanders S, Greenberg C, Cardwell S, Lemire E, MacLeod P, Hayden MR. Predictive, pre-natal and diagnostic genetic testing for Huntington's disease: the experience in Canada from 1987 to 2000. Clin. Genet. 63(6):462-75. (2003) PMID 12786753 235 Djoussé L, Knowlton B, Hayden M, Almqvist EW, Brinkman R, Ross C, Margolis R, Rosenblatt A, Durr A, Dode C, Morrison PJ, Novelletto A, Frontali M, Trent RJ, McCusker E, Gómez-Tortosa E, Mayo D, Jones R, Zanko A, Nance M, Abramson R, Suchowersky O, Paulsen J, Harrison M, Yang Q, Cupples LA, Gusella JF, MacDonald ME, Myers RH. Interaction of normal and expanded CAG repeat sizes influences age at onset of Huntington disease. Am. J. Med. Genet. A 119A(3):279-82. (2003) PMID 12784292 236 Bisoendial RJ, Hovingh GK, Levels JH, Lerch PG, Andresen I, Hayden MR, Kastelein JJ, Stroes ES. Restoration of endothelial function by increasing high-density lipoprotein in subjects with isolated low high-density lipoprotein. Circulation 107(23):2944-8. (2003) PMID 12771001 237 Singaraja RR, Brunham LR, Visscher H, Kastelein JJ, Hayden MR. Efflux and atherosclerosis: the clinical and biochemical impact of variations in the ABCA1 gene. Arterioscler. Thromb. Vasc. Biol. 23(8):1322-32. (2003) PMID 12763760 238 Hussain NK, Yamabhai M, Bhakar AL, Metzler M, Ferguson SS, Hayden MR, McPherson PS, Kay BK. A role for epsin N-terminal homology/AP180 N-terminal homology (ENTH/ANTH) domains in tubulin binding. J. Biol. Chem. 278(31):28823-30. (2003) PMID 12750376 239 Wellington CL, Brunham LR, Zhou S, Singaraja RR, Visscher H, Gelfer A, Ross C, James E, Liu G, Huber MT, Yang YZ, Parks RJ, Groen A, Fruchart-Najib J, Hayden MR. Alterations of plasma lipids in mice via adenoviral-mediated hepatic overexpression of human ABCA1. J. Lipid Res. 44(8):1470-80. (2003) PMID 12730295 240 Hovingh GK, Van Wijland MJ, Brownlie A, Bisoendial RJ, Hayden MR, Kastelein JJ, Groen AK. The role of the ABCA1 transporter and cholesterol efflux in familial hypoalphalipoproteinemia. J. Lipid Res. 44(6):1251-5. (2003) PMID 12700344 241 Squitieri F, Gellera C, Cannella M, Mariotti C, Cislaghi G, Rubinsztein DC, Almqvist EW, Turner D, Bachoud-Lévi AC, Simpson SA, Delatycki M, Maglione V, Hayden MR, Donato SD. Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course. Brain 126(Pt 4):946-55. (2003) PMID 12615650 242 Mulligan JD, Flowers MT, Tebon A, Bitgood JJ, Wellington C, Hayden MR, Attie AD. ABCA1 is essential for efficient basolateral cholesterol efflux during the absorption of dietary cholesterol in chickens. J. Biol. Chem. 278(15):13356-66. (2003) PMID 12551945 243 Gros-Louis F, Meijer IA, Hand CK, Dubé MP, MacGregor DL, Seni MH, Devon RS, Hayden MR, Andermann F, Andermann E, Rouleau GA. An ALS2 gene mutation causes hereditary spastic paraplegia in a Pakistani kindred. Ann. Neurol. 53(1):144-5. (2003) PMID 12509863 244 Laurent C, Niehaus D, Bauché S, Levinson DF, Soubigou S, Pimstone S, Hayden M, Mbanga I, Emsley R, Deleuze JF, Mallet J. CAG repeat polymorphisms in KCNN3 (HSKCa3) and PPP2R2B show no association or linkage to schizophrenia. Am. J. Med. Genet. B Neuropsychiatr. Genet. 116B(1):45-50. (2003) PMID 12497613 245 Beever CL, Stephenson MD, Peñaherrera MS, Jiang RH, Kalousek DK, Hayden M, Field L, Brown CJ, Robinson WP. Skewed X-chromosome inactivation is associated with trisomy in women ascertained on the basis of recurrent spontaneous abortion or chromosomally abnormal pregnancies. Am. J. Hum. Genet. 72(2):399-407. (2003) PMID 12497247 246 Nucifora FC, Ellerby LM, Wellington CL, Wood JD, Herring WJ, Sawa A, Hayden MR, Dawson VL, Dawson TM, Ross CA. Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity. J. Biol. Chem. 278(15):13047-55. (2003) PMID 12464607 247 Wellington CL, Ellerby LM, Leavitt BR, Roy S, Nicholson DW, Hayden MR. Huntingtin Proteolysis in Huntington disease Clinical Neuroscience Research 3(3):129-139. (2003) 248 Wellington CL, Yang YZ, Zhou S, Clee SM, Tan B, Hirano K, Zwarts K, Kwok A, Gelfer A, Marcil M, Newman S, Roomp K, Singaraja R, Collins J, Zhang LH, Groen AK, Hovingh K, Brownlie A, Tafuri S, Genest J, Kastelein JJ, Hayden MR. Truncation mutations in ABCA1 suppress normal upregulation of full-length ABCA1 by 9-cis-retinoic acid and 22-R-hydroxycholesterol. J. Lipid Res. 43(11):1939-49. (2002) PMID 12401893 249 Attie AD, Krauss RM, Gray-Keller MP, Brownlie A, Miyazaki M, Kastelein JJ, Lusis AJ, Stalenhoef AF, Stoehr JP, Hayden MR, Ntambi JM. Relationship between stearoyl-CoA desaturase activity and plasma triglycerides in human and mouse hypertriglyceridemia. J. Lipid Res. 43(11):1899-907. (2002) PMID 12401889 250 Singaraja RR, Hadano S, Metzler M, Givan S, Wellington CL, Warby S, Yanai A, Gutekunst CA, Leavitt BR, Yi H, Fichter K, Gan L, McCutcheon K, Chopra V, Michel J, Hersch SM, Ikeda JE, Hayden MR. HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum. Mol. Genet. 11(23):2815-28. (2002) PMID 12393793 251 Attie AD, Hamon Y, Brooks-Wilson AR, Gray-Keller MP, MacDonald ML, Rigot V, Tebon A, Zhang LH, Mulligan JD, Singaraja RR, Bitgood JJ, Cook ME, Kastelein JJ, Chimini G, Hayden MR. Identification and functional analysis of a naturally occurring E89K mutation in the ABCA1 gene of the WHAM chicken. J. Lipid Res. 43(10):1610-7. (2002) PMID 12364545 252 Wellington CL, Ellerby LM, Gutekunst CA, Rogers D, Warby S, Graham RK, Loubser O, van Raamsdonk J, Singaraja R, Yang YZ, Gafni J, Bredesen D, Hersch SM, Leavitt BR, Roy S, Nicholson DW, Hayden MR. Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. J. Neurosci. 22(18):7862-72. (2002) PMID 12223539 253 See RH, Caday-Malcolm RA, Singaraja RR, Zhou S, Silverston A, Huber MT, Moran J, James ER, Janoo R, Savill JM, Rigot V, Zhang LH, Wang M, Chimini G, Wellington CL, Tafuri SR, Hayden MR. Protein kinase A site-specific phosphorylation regulates ATP-binding cassette A1 (ABCA1)-mediated phospholipid efflux. J. Biol. Chem. 277(44):41835-42. (2002) PMID 12196520 254 Robitaille J, MacDonald ML, Kaykas A, Sheldahl LC, Zeisler J, Dubé MP, Zhang LH, Singaraja RR, Guernsey DL, Zheng B, Siebert LF, Hoskin-Mott A, Trese MT, Pimstone SN, Shastry BS, Moon RT, Hayden MR, Goldberg YP, Samuels ME. Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy. Nat. Genet. 32(2):326-30. (2002) PMID 12172548 255 Chan EY, Luthi-Carter R, Strand A, Solano SM, Hanson SA, DeJohn MM, Kooperberg C, Chase KO, DiFiglia M, Young AB, Leavitt BR, Cha JH, Aronin N, Hayden MR, Olson JM. Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease. Hum. Mol. Genet. 11(17):1939-51. (2002) PMID 12165556 256 Ariano MA, Aronin N, Difiglia M, Tagle DA, Sibley DR, Leavitt BR, Hayden MR, Levine MS. Striatal neurochemical changes in transgenic models of Huntington's disease. J. Neurosci. Res. 68(6):716-29. (2002) PMID 12111832 257 Singaraja RR, Fievet C, Castro G, James ER, Hennuyer N, Clee SM, Bissada N, Choy JC, Fruchart JC, McManus BM, Staels B, Hayden MR. Increased ABCA1 activity protects against atherosclerosis. J. Clin. Invest. 110(1):35-42. (2002) PMID 12093886 258 Panov AV, Gutekunst CA, Leavitt BR, Hayden MR, Burke JR, Strittmatter WJ, Greenamyre JT. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat. Neurosci. 5(8):731-6. (2002) PMID 12089530 259 Bu L, Jin Y, Shi Y, Chu R, Ban A, Eiberg H, Andres L, Jiang H, Zheng G, Qian M, Cui B, Xia Y, Liu J, Hu L, Zhao G, Hayden MR, Kong X. Mutant DNA-binding domain of HSF4 is associated with autosomal dominant lamellar and Marner cataract. Nat. Genet. 31(3):276-8. (2002) PMID 12089525 260 Chan EY, Nasir J, Gutekunst CA, Coleman S, Maclean A, Maas A, Metzler M, Gertsenstein M, Ross CA, Nagy A, Hayden MR. Targeted disruption of Huntingtin-associated protein-1 (Hap1) results in postnatal death due to depressed feeding behavior. Hum. Mol. Genet. 11(8):945-59. (2002) PMID 11971876 261 Zwarts KY, Clee SM, Zwinderman AH, Engert JC, Singaraja R, Loubser O, James E, Roomp K, Hudson TJ, Jukema JW, Kastelein JJ, Hayden MR. ABCA1 regulatory variants influence coronary artery disease independent of effects on plasma lipid levels. Clin. Genet. 61(2):115-25. (2002) PMID 11940086 262 Zeron MM, Hansson O, Chen N, Wellington CL, Leavitt BR, Brundin P, Hayden MR, Raymond LA. Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron 33(6):849-60. (2002) PMID 11906693 263 Wellington CL, Walker EK, Suarez A, Kwok A, Bissada N, Singaraja R, Yang YZ, Zhang LH, James E, Wilson JE, Francone O, McManus BM, Hayden MR. ABCA1 mRNA and protein distribution patterns predict multiple different roles and levels of regulation. Lab. Invest. 82(3):273-83. (2002) PMID 11896206 264 Peterson J, Ayyobi AF, Ma Y, Henderson H, Reina M, Deeb SS, Santamarina-Fojo S, Hayden MR, Brunzell JD. Structural and functional consequences of missense mutations in exon 5 of the lipoprotein lipase gene. J. Lipid Res. 43(3):398-406. (2002) PMID 11893776 265 Legendre-Guillemin V, Metzler M, Charbonneau M, Gan L, Chopra V, Philie J, Hayden MR, McPherson PS. HIP1 and HIP12 display differential binding to F-actin, AP2, and clathrin. Identification of a novel interaction with clathrin light chain. J. Biol. Chem. 277(22):19897-904. (2002) PMID 11889126 266 van Dam MJ, de Groot E, Clee SM, Hovingh GK, Roelants R, Brooks-Wilson A, Zwinderman AH, Smit AJ, Smelt AH, Groen AK, Hayden MR, Kastelein JJ. Association between increased arterial-wall thickness and impairment in ABCA1-driven cholesterol efflux: an observational study. Lancet 359(9300):37-42. (2002) PMID 11809185 267 Gervais FG, Singaraja R, Xanthoudakis S, Gutekunst CA, Leavitt BR, Metzler M, Hackam AS, Tam J, Vaillancourt JP, Houtzager V, Rasper DM, Roy S, Hayden MR, Nicholson DW. Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat. Cell Biol. 4(2):95-105. (2002) PMID 11788820 268 Nishida Y, Hirano K, Tsukamoto K, Nagano M, Ikegami C, Roomp K, Ishihara M, Sakane N, Zhang Z, Tsujii Ki K, Matsuyama A, Ohama T, Matsuura F, Ishigami M, Sakai N, Hiraoka H, Hattori H, Wellington C, Yoshida Y, Misugi S, Hayden MR, Egashira T, Yamashita S, Matsuzawa Y. Expression and functional analyses of novel mutations of ATP-binding cassette transporter-1 in Japanese patients with high-density lipoprotein deficiency. Biochem. Biophys. Res. Commun. 290(2):713-21. (2002) PMID 11785958 269 Cepeda C, Ariano MA, Calvert CR, Flores-Hernández J, Chandler SH, Leavitt BR, Hayden MR, Levine MS. NMDA receptor function in mouse models of Huntington disease. J. Neurosci. Res. 66(4):525-39. (2001) PMID 11746372 270 Squitieri F, Cannella M, Giallonardo P, Maglione V, Mariotti C, Hayden MR. Onset and pre-onset studies to define the Huntington's disease natural history. Brain Res. Bull. 56(3-4):233-8. (2001) PMID 11719256 271 Attie AD, Kastelein JP, Hayden MR. Pivotal role of ABCA1 in reverse cholesterol transport influencing HDL levels and susceptibility to atherosclerosis. J. Lipid Res. 42(11):1717-26. (2001) PMID 11714841 272 Parker JA, Connolly JB, Wellington C, Hayden M, Dausset J, Neri C. Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Proc. Natl. Acad. Sci. U.S.A. 98(23):13318-23. (2001) PMID 11687635 273 Clee SM, Loubser O, Collins J, Kastelein JJ, Hayden MR. The LPL S447X cSNP is associated with decreased blood pressure and plasma triglycerides, and reduced risk of coronary artery disease. Clin. Genet. 60(4):293-300. (2001) PMID 11683775 274 Almqvist EW, Elterman DS, MacLeod PM, Hayden MR. High incidence rate and absent family histories in one quarter of patients newly diagnosed with Huntington disease in British Columbia. Clin. Genet. 60(3):198-205. (2001) PMID 11595021 275 Hadano S, Hand CK, Osuga H, Yanagisawa Y, Otomo A, Devon RS, Miyamoto N, Showguchi-Miyata J, Okada Y, Singaraja R, Figlewicz DA, Kwiatkowski T, Hosler BA, Sagie T, Skaug J, Nasir J, Brown RH, Scherer SW, Rouleau GA, Hayden MR, Ikeda JE. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat. Genet. 29(2):166-73. (2001) PMID 11586298 276 Paulsen JS, Zhao H, Stout JC, Brinkman RR, Guttman M, Ross CA, Como P, Manning C, Hayden MR, Shoulson I, . Clinical markers of early disease in persons near onset of Huntington's disease. Neurology 57(4):658-62. (2001) PMID 11524475 277 Metzler M, Legendre-Guillemin V, Gan L, Chopra V, Kwok A, McPherson PS, Hayden MR. HIP1 functions in clathrin-mediated endocytosis through binding to clathrin and adaptor protein 2. J. Biol. Chem. 276(42):39271-6. (2001) PMID 11517213 278 Peel AL, Rao RV, Cottrell BA, Hayden MR, Ellerby LM, Bredesen DE. Double-stranded RNA-dependent protein kinase, PKR, binds preferentially to Huntington's disease (HD) transcripts and is activated in HD tissue. Hum. Mol. Genet. 10(15):1531-8. (2001) PMID 11468270 279 Rosenblatt A, Brinkman RR, Liang KY, Almqvist EW, Margolis RL, Huang CY, Sherr M, Franz ML, Abbott MH, Hayden MR, Ross CA. Familial influence on age of onset among siblings with Huntington disease. Am. J. Med. Genet. 105(5):399-403. (2001) PMID 11449389 280 Singaraja RR, Bocher V, James ER, Clee SM, Zhang LH, Leavitt BR, Tan B, Brooks-Wilson A, Kwok A, Bissada N, Yang YZ, Liu G, Tafuri SR, Fievet C, Wellington CL, Staels B, Hayden MR. Human ABCA1 BAC transgenic mice show increased high density lipoprotein cholesterol and ApoAI-dependent efflux stimulated by an internal promoter containing liver X receptor response elements in intron 1. J. Biol. Chem. 276(36):33969-79. (2001) PMID 11423537 281 Zuccato C, Ciammola A, Rigamonti D, Leavitt BR, Goffredo D, Conti L, MacDonald ME, Friedlander RM, Silani V, Hayden MR, Timmusk T, Sipione S, Cattaneo E. Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 293(5529):493-8. (2001) PMID 11408619 282 McGladdery SH, Pimstone SN, Clee SM, Bowden JF, Hayden MR, Frohlich JJ. Common mutations in the lipoprotein lipase gene (LPL): effects on HDL-cholesterol levels in a Chinese Canadian population. Atherosclerosis 156(2):401-7. (2001) PMID 11395037 283 Emsley RA, Niehaus DJ, Mbanga NI, Oosthuizen PP, Stein DJ, Maritz JS, Pimstone SN, Hayden MR, Laurent C, Deleuze JF, Mallet J. The factor structure for positive and negative symptoms in South African Xhosa patients with schizophrenia. Schizophr. Res. 47(2-3):149-57. (2001) PMID 11278132 284 Otto CJ, Almqvist E, Hayden MR, Andrew SE. The "flap" endonuclease gene FEN1 is excluded as a candidate gene implicated in the CAG repeat expansion underlying Huntington disease. Clin. Genet. 59(2):122-7. (2001) PMID 11260214 285 Clee SM, Zwinderman AH, Engert JC, Zwarts KY, Molhuizen HO, Roomp K, Jukema JW, van Wijland M, van Dam M, Hudson TJ, Brooks-Wilson A, Genest J, Kastelein JJ, Hayden MR. Common genetic variation in ABCA1 is associated with altered lipoprotein levels and a modified risk for coronary artery disease. Circulation 103(9):1198-205. (2001) PMID 11238261 286 Falush D, Almqvist EW, Brinkmann RR, Iwasa Y, Hayden MR. Measurement of mutational flow implies both a high new-mutation rate for Huntington disease and substantial underascertainment of late-onset cases. Am. J. Hum. Genet. 68(2):373-85. (2001) PMID 11225602 287 Backus RC, Ginzinger DG, Ashbourne Excoffon KJ, Clee SM, Hayden MR, Eckel RH, Hickman MA, Rogers QR. Maternal expression of functional lipoprotein lipase and effects on body fat mass and body condition scores of mature cats with lipoprotein lipase deficiency. Am. J. Vet. Res. 62(2):264-9. (2001) PMID 11212037 288 Holbert S, Denghien I, Kiechle T, Rosenblatt A, Wellington C, Hayden MR, Margolis RL, Ross CA, Dausset J, Ferrante RJ, Néri C. The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc. Natl. Acad. Sci. U.S.A. 98(4):1811-6. (2001) PMID 11172033 289 Hadano S, Yanagisawa Y, Skaug J, Fichter K, Nasir J, Martindale D, Koop BF, Scherer SW, Nicholson DW, Rouleau GA, Ikeda J, Hayden MR. Cloning and characterization of three novel genes, ALS2CR1, ALS2CR2, and ALS2CR3, in the juvenile amyotrophic lateral sclerosis (ALS2) critical region at chromosome 2q33-q34: candidate genes for ALS2. Genomics 71(2):200-13. (2001) PMID 11161814 290 Zeron MM, Chen N, Moshaver A, Lee AT, Wellington CL, Hayden MR, Raymond LA. Mutant huntingtin enhances excitotoxic cell death. Mol. Cell. Neurosci. 17(1):41-53. (2001) PMID 11161468 291 Leavitt BR, Guttman JA, Hodgson JG, Kimel GH, Singaraja R, Vogl AW, Hayden MR. Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo. Am. J. Hum. Genet. 68(2):313-24. (2001) PMID 11133364 292 Wellington CL, Leavitt BR, Hayden MR. Huntington disease: new insights on the role of huntingtin cleavage. J. Neural Transm. Suppl. (2000) PMID 11128600 293 Tsuang D, Almqvist EW, Lipe H, Strgar F, DiGiacomo L, Hoff D, Eugenio C, Hayden MR, Bird TD. Familial aggregation of psychotic symptoms in Huntington's disease. Am J Psychiatry 157(12):1955-9. (2000) PMID 11097960 294 Clee SM, Kastelein JJ, van Dam M, Marcil M, Roomp K, Zwarts KY, Collins JA, Roelants R, Tamasawa N, Stulc T, Suda T, Ceska R, Boucher B, Rondeau C, DeSouich C, Brooks-Wilson A, Molhuizen HO, Frohlich J, Genest J, Hayden MR. Age and residual cholesterol efflux affect HDL cholesterol levels and coronary artery disease in ABCA1 heterozygotes. J. Clin. Invest. 106(10):1263-70. (2000) PMID 11086027 295 Chopra VS, Metzler M, Rasper DM, Engqvist-Goldstein AE, Singaraja R, Gan L, Fichter KM, McCutcheon K, Drubin D, Nicholson DW, Hayden MR. HIP12 is a non-proapoptotic member of a gene family including HIP1, an interacting protein with huntingtin. Mamm. Genome 11(11):1006-15. (2000) PMID 11063258 296 Kastelein JJ, Jukema JW, Zwinderman AH, Clee S, van Boven AJ, Jansen H, Rabelink TJ, Peters RJ, Lie KI, Liu G, Bruschke AV, Hayden MR. Lipoprotein lipase activity is associated with severity of angina pectoris. REGRESS Study Group. Circulation 102(14):1629-33. (2000) PMID 11015339 297 Hackam AS, Yassa AS, Singaraja R, Metzler M, Gutekunst CA, Gan L, Warby S, Wellington CL, Vaillancourt J, Chen N, Gervais FG, Raymond L, Nicholson DW, Hayden MR. Huntingtin interacting protein 1 induces apoptosis via a novel caspase-dependent death effector domain. J. Biol. Chem. 275(52):41299-308. (2000) PMID 11007801 298 Nasir J, Lafuente MJ, Duan K, Colomer V, Engelender S, Ingersoll R, Margolis RL, Ross CA, Hayden MR. Human huntingtin-associated protein (HAP-1) gene: genomic organisation and an intragenic polymorphism. Gene 254(1-2):181-7. (2000) PMID 10974549 299 Clarke G, Collins RA, Leavitt BR, Andrews DF, Hayden MR, Lumsden CJ, McInnes RR. A one-hit model of cell death in inherited neuronal degenerations. Nature 406(6792):195-9. (2000) PMID 10910361 300 Rigamonti D, Bauer JH, De-Fraja C, Conti L, Sipione S, Sciorati C, Clementi E, Hackam A, Hayden MR, Li Y, Cooper JK, Ross CA, Govoni S, Vincenz C, Cattaneo E. Wild-type huntingtin protects from apoptosis upstream of caspase-3. J. Neurosci. 20(10):3705-13. (2000) PMID 10804212 301 Wellington CL, Singaraja R, Ellerby L, Savill J, Roy S, Leavitt B, Cattaneo E, Hackam A, Sharp A, Thornberry N, Nicholson DW, Bredesen DE, Hayden MR. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells. J. Biol. Chem. 275(26):19831-8. (2000) PMID 10770929 302 Clee SM, Bissada N, Miao F, Miao L, Marais AD, Henderson HE, Steures P, McManus J, McManus B, LeBoeuf RC, Kastelein JJ, Hayden MR. Plasma and vessel wall lipoprotein lipase have different roles in atherosclerosis. J. Lipid Res. 41(4):521-31. (2000) PMID 10744772 303 Metzler M, Helgason CD, Dragatsis I, Zhang T, Gan L, Pineault N, Zeitlin SO, Humphries RK, Hayden MR. Huntingtin is required for normal hematopoiesis. Hum. Mol. Genet. 9(3):387-94. (2000) PMID 10655548 304 Liu G, Ashbourne Excoffon KJ, Wilson JE, McManus BM, Rogers QR, Miao L, Kastelein JJ, Lewis ME, Hayden MR. Phenotypic correction of feline lipoprotein lipase deficiency by adenoviral gene transfer. Hum. Gene Ther. 11(1):21-32. (2000) PMID 10646636 305 Leavitt BR, Wellington CL, Hayden MR. Recent insights into the molecular pathogenesis of Huntington disease. Semin Neurol 19(4):385-95. (1999) PMID 10716661 306 Kastelein JJ, Ordovas JM, Wittekoek ME, Pimstone SN, Wilson WF, Gagné SE, Larson MG, Schaefer EJ, Boer JM, Gerdes C, Hayden MR. Two common mutations (D9N, N291S) in lipoprotein lipase: a cumulative analysis of their influence on plasma lipids and lipoproteins in men and women. Clin. Genet. 56(4):297-305. (1999) PMID 10636448 307 Engqvist-Goldstein AE, Kessels MM, Chopra VS, Hayden MR, Drubin DG. An actin-binding protein of the Sla2/Huntingtin interacting protein 1 family is a novel component of clathrin-coated pits and vesicles. J. Cell Biol. 147(7):1503-18. (1999) PMID 10613908 308 Wittekoek ME, Moll E, Pimstone SN, Trip MD, Lansberg PJ, Defesche JC, van Doormaal JJ, Hayden MR, Kastelein JJ. A frequent mutation in the lipoprotein lipase gene (D9N) deteriorates the biochemical and clinical phenotype of familial hypercholesterolemia. Arterioscler. Thromb. Vasc. Biol. 19(11):2708-13. (1999) PMID 10559015 309 Marcil M, Brooks-Wilson A, Clee SM, Roomp K, Zhang LH, Yu L, Collins JA, van Dam M, Molhuizen HO, Loubster O, Ouellette BF, Sensen CW, Fichter K, Mott S, Denis M, Boucher B, Pimstone S, Genest J, Kastelein JJ, Hayden MR. Mutations in the ABC1 gene in familial HDL deficiency with defective cholesterol efflux. Lancet 354(9187):1341-6. (1999) PMID 10533863 310 Kremer B, Clark CM, Almqvist EW, Raymond LA, Graf P, Jacova C, Mezei M, Hardy MA, Snow B, Martin W, Hayden MR. Influence of lamotrigine on progression of early Huntington disease: a randomized clinical trial. Neurology 53(5):1000-11. (1999) PMID 10496259 311 Gagné SE, Larson MG, Pimstone SN, Schaefer EJ, Kastelein JJ, Wilson PW, Ordovas JM, Hayden MR. A common truncation variant of lipoprotein lipase (Ser447X) confers protection against coronary heart disease: the Framingham Offspring Study. Clin. Genet. 55(6):450-4. (1999) PMID 10450862 312 Hackam AS, Hodgson JG, Singaraja R, Zhang T, Gan L, Gutekunst CA, Hersch SM, Hayden MR. Evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease in cell culture and in transgenic mice expressing mutant huntingtin. Philos. Trans. R. Soc. Lond., B, Biol. Sci. 354(1386):1047-55. (1999) PMID 10434304 313 Brooks-Wilson A, Marcil M, Clee SM, Zhang LH, Roomp K, van Dam M, Yu L, Brewer C, Collins JA, Molhuizen HO, Loubser O, Ouelette BF, Fichter K, Ashbourne-Excoffon KJ, Sensen CW, Scherer S, Mott S, Denis M, Martindale D, Frohlich J, Morgan K, Koop B, Pimstone S, Kastelein JJ, Genest J, Hayden MR. Mutations in ABC1 in Tangier disease and familial high-density lipoprotein deficiency. Nat. Genet. 22(4):336-45. (1999) PMID 10431236 314 Hodgson JG, Agopyan N, Gutekunst CA, Leavitt BR, LePiane F, Singaraja R, Smith DJ, Bissada N, McCutcheon K, Nasir J, Jamot L, Li XJ, Stevens ME, Rosemond E, Roder JC, Phillips AG, Rubin EM, Hersch SM, Hayden MR. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23(1):181-92. (1999) PMID 10402204 315 Hadano S, Nasir J, Nichol K, Rasper DM, Vaillancourt JP, Sherer SW, Beatty BG, Ikeda JE, Nicholson DW, Hayden MR. Genomic organization of the human caspase-9 gene on Chromosome 1p36. 1-p36.3. Mamm. Genome 10(7):757-60. (1999) PMID 10384055 316 Kim M, Velier J, Chase K, Laforet G, Kalchman MA, Hayden MR, Won L, Heller A, Aronin N, Difiglia M. Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin. Neuroscience 89(4):1159-67. (1999) PMID 10362304 317 Bruland O, Almqvist EW, Goldberg YP, Boman H, Hayden MR, Knappskog PM. Accurate determination of the number of CAG repeats in the Huntington disease gene using a sequence-specific internal DNA standard. Clin. Genet. 55(3):198-202. (1999) PMID 10334474 318 Chen N, Luo T, Wellington C, Metzler M, McCutcheon K, Hayden MR, Raymond LA. Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin. J. Neurochem. 72(5):1890-8. (1999) PMID 10217265 319 Almqvist EW, Bloch M, Brinkman R, Craufurd D, Hayden MR. A worldwide assessment of the frequency of suicide, suicide attempts, or psychiatric hospitalization after predictive testing for Huntington disease. Am. J. Hum. Genet. 64(5):1293-304. (1999) PMID 10205260 320 Henderson HE, Kastelein JJ, Zwinderman AH, Gagné E, Jukema JW, Reymer PW, Groenemeyer BE, Lie KI, Bruschke AV, Hayden MR, Jansen H. Lipoprotein lipase activity is decreased in a large cohort of patients with coronary artery disease and is associated with changes in lipids and lipoproteins. J. Lipid Res. 40(4):735-43. (1999) PMID 10191298 321 Ginzinger DG, Clee SM, Dallongeville J, Lewis ME, Henderson HE, Bauje E, Rogers QR, Jensen DR, Eckel RH, Dyer R, Innis S, Jones B, Fruchart JC, Hayden MR. Lipid and lipoprotein analysis of cats with lipoprotein lipase deficiency. Eur. J. Clin. Invest. 29(1):17-26. (1999) PMID 10092984 322 Nasir J, Maclean A, Engelender S, Duan K, Margolis RL, Kleiderlein JJ, Ross CA, Hayden MR. Chromosomal localization of the Huntingtin associated protein (HAP-1) gene in mouse and humans with radiation hybrid and interspecific backcross mapping. Mamm. Genome 10(4):397-8. (1999) PMID 10087300 323 Ellerby LM, Andrusiak RL, Wellington CL, Hackam AS, Propp SS, Wood JD, Sharp AH, Margolis RL, Ross CA, Salvesen GS, Hayden MR, Bredesen DE. Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity. J. Biol. Chem. 274(13):8730-6. (1999) PMID 10085113 324 O'Kusky JR, Nasir J, Cicchetti F, Parent A, Hayden MR. Neuronal degeneration in the basal ganglia and loss of pallido-subthalamic synapses in mice with targeted disruption of the Huntington's disease gene. Brain Res. 818(2):468-79. (1999) PMID 10082833 325 Metzler M, Chen N, Helgason CD, Graham RK, Nichol K, McCutcheon K, Nasir J, Humphries RK, Raymond LA, Hayden MR. Life without huntingtin: normal differentiation into functional neurons. J. Neurochem. 72(3):1009-18. (1999) PMID 10037472 326 Hadano S, Nichol K, Brinkman RR, Nasir J, Martindale D, Koop BF, Nicholson DW, Scherer SW, Ikeda JE, Hayden MR. A yeast artificial chromosome-based physical map of the juvenile amyotrophic lateral sclerosis (ALS2) critical region on human chromosome 2q33-q34. Genomics 55(1):106-12. (1999) PMID 9889004 327 Hackam AS, Singaraja R, Zhang T, Gan L, Hayden MR. In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease. Hum. Mol. Genet. 8(1):25-33. (1999) PMID 9887328 328 Ellerby LM, Hackam AS, Propp SS, Ellerby HM, Rabizadeh S, Cashman NR, Trifiro MA, Pinsky L, Wellington CL, Salvesen GS, Hayden MR, Bredesen DE. Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity. J. Neurochem. 72(1):185-95. (1999) PMID 9886069 329 Liu G, Excoffon KJ, Wilson JE, McManus BM, Miao L, Benoit P, Duverger N, Branellec D, Denefle P, Hayden MR, Lewis ME. Enhanced lipolysis in normal mice expressing liver-derived human lipoprotein lipase after adenoviral gene transfer. Clin Invest Med 21(4-5):172-85. (1999) PMID 9800066 330 Rasper DM, Vaillancourt JP, Hadano S, Houtzager VM, Seiden I, Keen SL, Tawa P, Xanthoudakis S, Nasir J, Martindale D, Koop BF, Peterson EP, Thornberry NA, Huang J, MacPherson DP, Black SC, Hornung F, Lenardo MJ, Hayden MR, Roy S, Nicholson DW. Cell death attenuation by 'Usurpin', a mammalian DED-caspase homologue that precludes caspase-8 recruitment and activation by the CD-95 (Fas, APO-1) receptor complex. Cell Death Differ. 5(4):271-88. (1998) PMID 10200473 331 Hayden MR. In vitro and in vivo models for Huntington disease: lessons for the polyglutamine expansion disorders. Pathol. Biol. 46(9):695-6. (1998) PMID 9885820 332 Xiang F, Almqvist EW, Huq M, Lundin A, Hayden MR, Edström L, Anvret M, Zhang Z. A Huntington disease-like neurodegenerative disorder maps to chromosome 20p. Am. J. Hum. Genet. 63(5):1431-8. (1998) PMID 9792871 333 Henderson HE, Bijvoet SM, Mannens MA, Bruin T, Erkelens DW, Hayden MR, Kastelein JJ. Ile225Thr loop mutation in the lipoprotein lipase (LPL) gene is a de novo event. Am. J. Med. Genet. 78(4):313-6. (1998) PMID 9714430 334 Nasir J, Duan K, Nichol K, Engelender S, Ashworth R, Colomer V, Thomas S, Disteche CM, Hayden MR, Ross CA. Gene structure and map location of the murine homolog of the Huntington-associated protein, Hap1. Mamm. Genome 9(7):565-70. (1998) PMID 9657855 335 Hackam AS, Singaraja R, Wellington CL, Metzler M, McCutcheon K, Zhang T, Kalchman M, Hayden MR. The influence of huntingtin protein size on nuclear localization and cellular toxicity. J. Cell Biol. 141(5):1097-105. (1998) PMID 9606203 336 Kastelein JJ, Groenemeyer BE, Hallman DM, Henderson H, Reymer PW, Gagné SE, Jansen H, Seidell JC, Kromhout D, Jukema JW, Bruschke AV, Boerwinkle E, Hayden MR. The Asn9 variant of lipoprotein lipase is associated with the -93G promoter mutation and an increased risk of coronary artery disease. The Regress Study Group. Clin. Genet. 53(1):27-33. (1998) PMID 9550358 337 Wellington CL, Ellerby LM, Hackam AS, Margolis RL, Trifiro MA, Singaraja R, McCutcheon K, Salvesen GS, Propp SS, Bromm M, Rowland KJ, Zhang T, Rasper D, Roy S, Thornberry N, Pinsky L, Kakizuka A, Ross CA, Nicholson DW, Bredesen DE, Hayden MR. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J. Biol. Chem. 273(15):9158-67. (1998) PMID 9535906 338 Wittekoek ME, Pimstone SN, Reymer PW, Feuth L, Botma GJ, Defesche JC, Prins M, Hayden MR, Kastelein JJ. A common mutation in the lipoprotein lipase gene (N291S) alters the lipoprotein phenotype and risk for cardiovascular disease in patients with familial hypercholesterolemia. Circulation 97(8):729-35. (1998) PMID 9498535 339 Henderson H, Leisegang F, Hassan F, Hayden M, Marais D. A novel Glu421Lys substitution in the lipoprotein lipase gene in pregnancy-induced hypertriglyceridemic pancreatitis. Clin. Chim. Acta 269(1):1-12. (1998) PMID 9498099 340 Pimstone SN, Sun XM, du Souich C, Frohlich JJ, Hayden MR, Soutar AK. Phenotypic variation in heterozygous familial hypercholesterolemia: a comparison of Chinese patients with the same or similar mutations in the LDL receptor gene in China or Canada. Arterioscler. Thromb. Vasc. Biol. 18(2):309-15. (1998) PMID 9484998 341 Martindale D, Hackam A, Wieczorek A, Ellerby L, Wellington C, McCutcheon K, Singaraja R, Kazemi-Esfarjani P, Devon R, Kim SU, Bredesen DE, Tufaro F, Hayden MR. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat. Genet. 18(2):150-4. (1998) PMID 9462744 342 Julien P, Gagné C, Murthy MR, Lévesque G, Moorjani S, Cadelis F, Hayden MR, Lupien PJ. Dyslipidemias associated with heterozygous lipoprotein lipase mutations in the French-Canadian population. Hum. Mutat. Suppl 1:S148-53. (1998) PMID 9452071 343 Foubert L, De Gennes JL, Benlian P, Truffert J, Miao L, Hayden MR. Compound heterozygosity for frameshift mutations in the gene for lipoprotein lipase in a patient with early-onset chylomicronemia. Hum. Mutat. Suppl 1:S141-4. (1998) PMID 9452069 344 Abdel-Wareth LO, Pimstone SN, Lagarde JP, Raisonnier A, Benlian P, Pritchard H, Hayden MR, Frohlich JJ. Familial defective apolipoprotein B-100 in hypercholesterolemic Chinese Canadians: identification of a unique haplotype of the apolipoprotein B-100 allele. Atherosclerosis 135(2):181-5. (1997) PMID 9430367 345 Ehrenborg E, Clee SM, Pimstone SN, Reymer PW, Benlian P, Hoogendijk CF, Davis HJ, Bissada N, Miao L, Gagné SE, Greenberg LJ, Henry R, Henderson H, Ordovas JM, Schaefer EJ, Kastelein JJ, Kotze MJ, Hayden MR. Ethnic variation and in vivo effects of the -93t-->g promoter variant in the lipoprotein lipase gene. Arterioscler. Thromb. Vasc. Biol. 17(11):2672-8. (1997) PMID 9409241 346 Excoffon KJ, Liu G, Miao L, Wilson JE, McManus BM, Semenkovich CF, Coleman T, Benoit P, Duverger N, Branellec D, Denefle P, Hayden MR, Lewis ME. Correction of hypertriglyceridemia and impaired fat tolerance in lipoprotein lipase-deficient mice by adenovirus-mediated expression of human lipoprotein lipase. Arterioscler. Thromb. Vasc. Biol. 17(11):2532-9. (1997) PMID 9409224 347 Ginzinger DG, Wilson JE, Redenbach D, Lewis ME, Clee SM, Excoffon KJ, Rogers QR, Hayden MR, McManus BM. Diet-induced atherosclerosis in the domestic cat. Lab. Invest. 77(5):409-19. (1997) PMID 9389784 348 Almqvist E, Adam S, Bloch M, Fuller A, Welch P, Eisenberg D, Whelan D, Macgregor D, Meschino W, Hayden MR. Risk reversals in predictive testing for Huntington disease. Am. J. Hum. Genet. 61(4):945-52. (1997) PMID 9382108 349 Clee SM, Zhang H, Bissada N, Miao L, Ehrenborg E, Benlian P, Shen GX, Angel A, LeBoeuf RC, Hayden MR. Relationship between lipoprotein lipase and high density lipoprotein cholesterol in mice: modulation by cholesteryl ester transfer protein and dietary status. J. Lipid Res. 38(10):2079-89. (1997) PMID 9374130 350 Foubert L, Bruin T, De Gennes JL, Ehrenborg E, Furioli J, Kastelein J, Benlian P, Hayden M. A single Ser259Arg mutation in the gene for lipoprotein lipase causes chylomicronemia in Moroccans of Berber ancestry. Hum. Mutat. 10(3):179-85. (1997) PMID 9298816 351 Foubert L, De Gennes JL, Lagarde JP, Ehrenborg E, Raisonnier A, Girardet JP, Hayden MR, Benlian P. Assessment of French patients with LPL deficiency for French Canadian mutations. J. Med. Genet. 34(8):672-5. (1997) PMID 9279761 352 Wellington CL, Hayden MR. Of molecular interactions, mice and mechanisms: new insights into Huntington's disease. Curr. Opin. Neurol. 10(4):291-8. (1997) PMID 9266152 353 Nasir J, Theilmann JL, Vaillancourt JP, Munday NA, Ali A, Scherer S, Beatty B, Nicholson DW, Hayden MR. Interleukin-1beta-converting enzyme (ICE) and related cell death genes ICErel-II and ICErel-III map to the same PAC clone at band 11q22.2-22.3. Mamm. Genome 8(8):611-3. (1997) PMID 9250871 354 Wellington CL, Brinkman RR, O'Kusky JR, Hayden MR. Toward understanding the molecular pathology of Huntington's disease. Brain Pathol. 7(3):979-1002. (1997) PMID 9217979 355 Burgess MM, Adam S, Bloch M, Hayden MR. Dilemmas of anonymous predictive testing for Huntington disease: privacy vs. optimal care. Am. J. Med. Genet. 71(2):197-201. (1997) PMID 9217222 356 Groenemeijer BE, Hallman MD, Reymer PW, Gagné E, Kuivenhoven JA, Bruin T, Jansen H, Lie KI, Bruschke AV, Boerwinkle E, Hayden MR, Kastelein JJ. Genetic variant showing a positive interaction with beta-blocking agents with a beneficial influence on lipoprotein lipase activity, HDL cholesterol, and triglyceride levels in coronary artery disease patients. The Ser447-stop substitution in the lipoprotein lipase gene. REGRESS Study Group. Circulation 95(12):2628-35. (1997) PMID 9193431 357 Pimstone SN, Defesche JC, Clee SM, Bakker HD, Hayden MR, Kastelein JJ. Differences in the phenotype between children with familial defective apolipoprotein B-100 and familial hypercholesterolemia. Arterioscler. Thromb. Vasc. Biol. 17(5):826-33. (1997) PMID 9157944 358 Brinkman RR, Mezei MM, Theilmann J, Almqvist E, Hayden MR. The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size. Am. J. Hum. Genet. 60(5):1202-10. (1997) PMID 9150168 359 Kalchman MA, Koide HB, McCutcheon K, Graham RK, Nichol K, Nishiyama K, Kazemi-Esfarjani P, Lynn FC, Wellington C, Metzler M, Goldberg YP, Kanazawa I, Gietz RD, Hayden MR. HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain. Nat. Genet. 16(1):44-53. (1997) PMID 9140394 360 Goellner GM, Tester D, Thibodeau S, Almqvist E, Goldberg YP, Hayden MR, McMurray CT. Different mechanisms underlie DNA instability in Huntington disease and colorectal cancer. Am. J. Hum. Genet. 60(4):879-90. (1997) PMID 9106534 361 Clark CM, Jacova C, Klonoff H, Kremer B, Hayden M, Paty D. Pathological association and dissociation of functional systems in multiple sclerosis and Huntington's disease. J Clin Exp Neuropsychol 19(1):63-76. (1997) PMID 9071642 362 Nielsen MS, Brejning J, García R, Zhang H, Hayden MR, Vilaró S, Gliemann J. Segments in the C-terminal folding domain of lipoprotein lipase important for binding to the low density lipoprotein receptor-related protein and to heparan sulfate proteoglycans. J. Biol. Chem. 272(9):5821-7. (1997) PMID 9038197 363 Liu G, Excoffon KJ, Benoit P, Ginzinger DG, Miao L, Ehrenborg E, Duverger N, Denefle PP, Hayden MR, Lewis ME. Efficient adenovirus-mediated ectopic gene expression of human lipoprotein lipase in human hepatic (HepG2) cells. Hum. Gene Ther. 8(2):205-14. (1997) PMID 9017424 364 Wiggins S, Green T, Adam S, Hayden MR. A long term (ca 5 years) prospective assessment of psychological consequences of predictive testing for Huntington disease (HD) Am. J. Hum. Genet. 59(4):A7. (1996) PMID 11644838 365 Marcil M, Boucher B, Gagné E, Davignon J, Hayden M, Genest J. Lack of association of the apolipoprotein A-I-C-III-A-IV gene XmnI and SstI polymorphisms and of the lipoprotein lipase gene mutations in familial combined hyperlipoproteinemia in French Canadian subjects. J. Lipid Res. 37(2):309-19. (1996) PMID 9026529 366 Bijvoet SM, Wiebusch H, Ma Y, Reymer PW, Bruin T, Bakker HD, Funke H, Assmann G, Hayden MR, Kastelein JJ. Compound heterozygosity for a known and a novel defect in the lipoprotein lipase gene (Asp250-->Asn; Ser251-->Cys) resulting in lipoprotein lipase (LPL) deficiency. 49(5):189-95. (1996) PMID 8973094 367 Hodgson JG, Smith DJ, McCutcheon K, Koide HB, Nishiyama K, Dinulos MB, Stevens ME, Bissada N, Nasir J, Kanazawa I, Disteche CM, Rubin EM, Hayden MR. Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype. Hum. Mol. Genet. 5(12):1875-85. (1996) PMID 8968738 368 Lawson K, Wiggins S, Green T, Adam S, Bloch M, Hayden MR. Adverse psychological events occurring in the first year after predictive testing for Huntington's disease. The Canadian Collaborative Study Predictive Testing. J. Med. Genet. 33(10):856-62. (1996) PMID 8933341 369 Defesche JC, Van Diermen DE, Hayden MR, Kastelein JP. Origin and migration of an Afrikaner founder mutation FHAfrikaner-2 (V408M) causing familial hypercholesterolemia. 10(1):1-10. (1996) PMID 8913716 370 Nasir J, Goldberg YP, Hayden MR. Huntington disease: new insights into the relationship between CAG expansion and disease. Hum. Mol. Genet. 5 Spec No:1431-5. (1996) PMID 8875248 371 Jukema JW, van Boven AJ, Groenemeijer B, Zwinderman AH, Reiber JH, Bruschke AV, Henneman JA, Molhoek GP, Bruin T, Jansen H, Gagné E, Hayden MR, Kastelein JJ. The Asp9 Asn mutation in the lipoprotein lipase gene is associated with increased progression of coronary atherosclerosis. REGRESS Study Group, Interuniversity Cardiology Institute, Utrecht, The Netherlands. Regression Growth Evaluation Statin Study. Circulation 94(8):1913-8. (1996) PMID 8873668 372 Bagdade JD, Ritter MC, Lithell H, Bassett D, Mailly F, Talmud P, Hayden MR. Reduced cholesteryl ester transfer in plasma of patients with lipoprotein lipase deficiency. J. Lipid Res. 37(8):1696-703. (1996) PMID 8864953 373 Pimstone SN, Clee SM, Gagné SE, Miao L, Zhang H, Stein EA, Hayden MR. A frequently occurring mutation in the lipoprotein lipase gene (Asn291Ser) results in altered postprandial chylomicron triglyceride and retinyl palmitate response in normolipidemic carriers. J. Lipid Res. 37(8):1675-84. (1996) PMID 8864951 374 Henderson HE, Hassan F, Marais D, Hayden MR. A new mutation destroying disulphide bridging in the C-terminal domain of lipoprotein lipase. Biochem. Biophys. Res. Commun. 227(1):189-94. (1996) PMID 8858123 375 Küpper M, Loftin U, von Bonin F, Gause A, Pfreundschuh M, Daus H, Trümper L. Single cell PCR for the analysis of Hodgkin's disease: four years later. Ann. Oncol. 7 Suppl 4:35-9. (1996) PMID 8836407 376 Goldberg YP, Kalchman MA, Metzler M, Nasir J, Zeisler J, Graham R, Koide HB, O'Kusky J, Sharp AH, Ross CA, Jirik F, Hayden MR. Absence of disease phenotype and intergenerational stability of the CAG repeat in transgenic mice expressing the human Huntington disease transcript. Hum. Mol. Genet. 5(2):177-85. (1996) PMID 8824873 377 Benlian P, De Gennes JL, Foubert L, Zhang H, Gagné SE, Hayden M. Premature atherosclerosis in patients with familial chylomicronemia caused by mutations in the lipoprotein lipase gene. N. Engl. J. Med. 335(12):848-54. (1996) PMID 8778602 378 Benlian P, Foubert L, Gagné E, Bernard L, De Gennes JL, Langlois S, Robinson W, Hayden M. Complete paternal isodisomy for chromosome 8 unmasked by lipoprotein lipase deficiency. Am. J. Hum. Genet. 59(2):431-6. (1996) PMID 8755931 379 Bijvoet S, Gagné SE, Moorjani S, Gagné C, Henderson HE, Fruchart JC, Dallongeville J, Alaupovic P, Prins M, Kastelein JJ, Hayden MR. Alterations in plasma lipoproteins and apolipoproteins before the age of 40 in heterozygotes for lipoprotein lipase deficiency. J. Lipid Res. 37(3):640-50. (1996) PMID 8728325 380 Kalchman MA, Graham RK, Xia G, Koide HB, Hodgson JG, Graham KC, Goldberg YP, Gietz RD, Pickart CM, Hayden MR. Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme. J. Biol. Chem. 271(32):19385-94. (1996) PMID 8702625 381 Goldberg YP, Nicholson DW, Rasper DM, Kalchman MA, Koide HB, Graham RK, Bromm M, Kazemi-Esfarjani P, Thornberry NA, Vaillancourt JP, Hayden MR. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nat. Genet. 13(4):442-9. (1996) PMID 8696339 382 Zhang H, Henderson H, Gagne SE, Clee SM, Miao L, Liu G, Hayden MR. Common sequence variants of lipoprotein lipase: standardized studies of in vitro expression and catalytic function. Biochim. Biophys. Acta 1302(2):159-66. (1996) PMID 8695666 383 . Unified Huntington's Disease Rating Scale: reliability and consistency. Huntington Study Group. Mov. Disord. 11(2):136-42. (1996) PMID 8684382 384 Shen GX, Zhang JY, Blanchard R, Zhang HF, Hayden M, McPherson R, Angel A. Analysis of cholesteryl ester transfer activity in adipose tissue. Int. J. Obes. Relat. Metab. Disord. 20 Suppl 3:S114-20. (1996) PMID 8680471 385 Rubinsztein DC, Leggo J, Coles R, Almqvist E, Biancalana V, Cassiman JJ, Chotai K, Connarty M, Crauford D, Curtis A, Curtis D, Davidson MJ, Differ AM, Dode C, Dodge A, Frontali M, Ranen NG, Stine OC, Sherr M, Abbott MH, Franz ML, Graham CA, Harper PS, Hedreen JC, Hayden MR. Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats. Am. J. Hum. Genet. 59(1):16-22. (1996) PMID 8659522 386 Ginzinger DG, Lewis ME, Ma Y, Jones BR, Liu G, Jones SD. A mutation in the lipoprotein lipase gene is the molecular basis of chylomicronemia in a colony of domestic cats. J. Clin. Invest. 97(5):1257-66. (1996) PMID 8636438 387 Lambert M, Lupien PJ, Gagné C, Lévy E, Blaichman S, Langlois S, Hayden M, Rose V, Clarke JT, Wolfe BM, Clarson C, Parsons H, Stephure DK, Potvin D, Lambert J. Treatment of familial hypercholesterolemia in children and adolescents: effect of lovastatin. Canadian Lovastatin in Children Study Group. Pediatrics 97(5):619-28. (1996) PMID 8628597 388 Lewis MES, Liu G, Ginzinger DG, Benoit P, Jones SD, Ashbourne KJD, Wilson JE, McManus BM, Duverger N, Denefle PP, Hayden MR. Gene-based Therapeutic Strategies for Human Lipoprotein Lipase (LPL) Deficiency: Rationale and Prospects for Alteration of Atherogenic Risk Transfusion and Apheresis Science 17(1):79-87. (1996) 389 Sedun K, Hayden MR. Letter to the Editor: Genetic Testing for Huntington Disease BC Medical Journal 38:317-318. (1996) 390 Krapp A, Zhang H, Ginzinger D, Liu MS, Lindberg A, Olivecrona G, Hayden MR, Beisiegel U. Structural features in lipoprotein lipase necessary for the mediation of lipoprotein uptake into cells. J. Lipid Res. 36(11):2362-73. (1995) PMID 8656074 391 Goldberg YP, McMurray CT, Zeisler J, Almqvist E, Sillence D, Richards F, Gacy AM, Buchanan J, Telenius H, Hayden MR. Increased instability of intermediate alleles in families with sporadic Huntington disease compared to similar sized intermediate alleles in the general population. Hum. Mol. Genet. 4(10):1911-8. (1995) PMID 8595415 392 Andrew SE, Hayden MR. Origins and evolution of Huntington disease chromosomes. Neurodegeneration 4(3):239-44. (1995) PMID 8581556 393 Reymer PW, Groenemeyer BE, Gagné E, Miao L, Appelman EE, Seidel JC, Kromhout D, Bijvoet SM, van de Oever K, Bruin T. A frequently occurring mutation in the lipoprotein lipase gene (Asn291Ser) contributes to the expression of familial combined hyperlipidemia. Hum. Mol. Genet. 4(9):1543-9. (1995) PMID 8541837 394 Britton JW, Uitti RJ, Ahlskog JE, Robinson RG, Kremer B, Hayden MR. Hereditary late-onset chorea without significant dementia: genetic evidence for substantial phenotypic variation in Huntington's disease. Neurology 45(3 Pt 1):443-7. (1995) PMID 7898693 395 Lin B, Nasir J, McDonald H, Graham R, Rommens JM, Goldberg YP, Hayden MR. Genomic organization of the human alpha-adducin gene and its alternately spliced isoforms. Genomics 25(1):93-9. (1995) PMID 7774961 396 Nasir J, Floresco SB, O'Kusky JR, Diewert VM, Richman JM, Zeisler J, Borowski A, Marth JD, Phillips AG, Hayden MR. Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 81(5):811-23. (1995) PMID 7774020 397 Estabrooks LL, Breg WR, Hayden MR, Ledbetter DH, Myers RM, Wyandt HE, Yang-Feng TL, Hirschhorn K. Summary of the 1993 ASHG ancillary meeting "recent research on chromosome 4p syndromes and genes". Am. J. Med. Genet. 55(4):453-8. (1995) PMID 7762585 398 Lin B, Nasir J, Kalchman MA, McDonald H, Zeisler J, Goldberg YP, Hayden MR. Structural analysis of the 5' region of mouse and human Huntington disease genes reveals conservation of putative promoter region and di- and trinucleotide polymorphisms. Genomics 25(3):707-15. (1995) PMID 7759106 399 Almqvist E, Spence N, Nichol K, Andrew SE, Vesa J, Peltonen L, Anvret M, Goto J, Kanazawa I, Goldberg YP. Ancestral differences in the distribution of the delta 2642 glutamic acid polymorphism is associated with varying CAG repeat lengths on normal chromosomes: insights into the genetic evolution of Huntington disease. Hum. Mol. Genet. 4(2):207-14. (1995) PMID 7757069 400 Telenius H, Almqvist E, Kremer B, Spence N, Squitieri F, Nichol K, Grandell U, Starr E, Benjamin C, Castaldo I. Somatic mosaicism in sperm is associated with intergenerational (CAG)n changes in Huntington disease. Hum. Mol. Genet. 4(2):189-95. (1995) PMID 7757066 401 Kremer B, Almqvist E, Theilmann J, Spence N, Telenius H, Goldberg YP, Hayden MR. Sex-dependent mechanisms for expansions and contractions of the CAG repeat on affected Huntington disease chromosomes. Am. J. Hum. Genet. 57(2):343-50. (1995) PMID 7668260 402 Reymer PW, Gagné E, Groenemeyer BE, Zhang H, Forsyth I, Jansen H, Seidell JC, Kromhout D, Lie KE, Kastelein J. A lipoprotein lipase mutation (Asn291Ser) is associated with reduced HDL cholesterol levels in premature atherosclerosis. Nat. Genet. 10(1):28-34. (1995) PMID 7647785 403 Leung PC, Squire J, Peng C, Fan N, Hayden MR, Olofsson JI. Mapping of the gonadotropin-releasing hormone (GnRH) receptor gene to human chromosome 4q21.2 by fluorescence in situ hybridization. Mamm. Genome 6(4):309-10. (1995) PMID 7613048 405 Pimstone SN, Gagné SE, Gagné C, Lupien PJ, Gaudet D, Williams RR, Kotze M, Reymer PW, Defesche JC, Kastelein JJ. Mutations in the gene for lipoprotein lipase. A cause for low HDL cholesterol levels in individuals heterozygous for familial hypercholesterolemia. Arterioscler. Thromb. Vasc. Biol. 15(10):1704-12. (1995) PMID 7583547 406 Zhang H, Reymer PW, Liu MS, Forsythe IJ, Groenemeyer BE, Frohlich J, Brunzell JD, Kastelein JJ, Hayden MR, Ma Y. Patients with apoE3 deficiency (E2/2, E3/2, and E4/2) who manifest with hyperlipidemia have increased frequency of an Asn 291-->Ser mutation in the human LPL gene. Arterioscler. Thromb. Vasc. Biol. 15(10):1695-703. (1995) PMID 7583546 407 Lewis ME, Forsythe IJ, Marth JD, Brunzell JD, Hayden MR, Humphries RK. Retroviral-mediated gene transfer and expression of human lipoprotein lipase in somatic cells. Hum. Gene Ther. 6(7):853-63. (1995) PMID 7578404 408 Lansberg PJ, Mitchel YB, Shapiro D, Kastelein JJ, Altman R, Jerums G, Bolzano K, Giannini S, Davignon J, DeWailly P. Long-term efficacy and tolerability of simvastatin in a large cohort of elderly hypercholesterolemic patients. Atherosclerosis 116(2):153-62. (1995) PMID 7575771 409 Copley TT, Wiggins S, Dufrasne S, Bloch M, Adam S, McKellin W, Hayden MR. Are we all of one mind? Clinicians' and patients' opinions regarding the development of a service protocol for predictive testing for Huntington disease. Canadian Collaborative Study for Predictive Testing for Huntington Disease. Am. J. Med. Genet. 58(1):59-69. (1995) PMID 7573158 410 Riess O, Thies U, Siedlaczck I, Potisek S, Graham R, Theilmann J, Grimm T, Epplen JT, Hayden MR. Precise mapping of the brain alpha 2-adrenergic receptor gene within chromosome 4p16. Genomics 19(2):298-302. (1994) PMID 8188260 411 Andrew SE, Goldberg YP, Kremer B, Squitieri F, Theilmann J, Zeisler J, Telenius H, Adam S, Almquist E, Anvret M. Huntington disease without CAG expansion: phenocopies or errors in assignment? Am. J. Hum. Genet. 54(5):852-63. (1994) PMID 8178825 412 Lin B, Nasir J, MacDonald H, Hutchinson G, Graham RK, Rommens JM, Hayden MR. Sequence of the murine Huntington disease gene: evidence for conservation, alternate splicing and polymorphism in a triplet (CCG) repeat [corrected] Hum. Mol. Genet. 3(1):85-92. (1994) PMID 8162057 413 Andrew SE, Goldberg YP, Theilmann J, Zeisler J, Hayden MR. A CCG repeat polymorphism adjacent to the CAG repeat in the Huntington disease gene: implications for diagnostic accuracy and predictive testing. Hum. Mol. Genet. 3(1):65-7. (1994) PMID 8162053 414 Kremer B, Goldberg P, Andrew SE, Theilmann J, Telenius H, Zeisler J, Squitieri F, Lin B, Bassett A, Almqvist E. A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats. N. Engl. J. Med. 330(20):1401-6. (1994) PMID 8159192 415 Liu MS, Jirik FR, LeBoeuf RC, Henderson H, Castellani LW, Lusis AJ, Ma Y, Forsythe IJ, Zhang H, Kirk E. Alteration of lipid profiles in plasma of transgenic mice expressing human lipoprotein lipase. J. Biol. Chem. 269(15):11417-24. (1994) PMID 8157673 416 Bijvoet SM, Bruin T, Tuzgöl S, Bakker HD, Hayden MR, Kastelein JJ. Homozygosity for a mutation in the lipoprotein lipase gene (Gly139-->Ser) causes chylomicronaemia in a boy of Spanish descent. Hum. Genet. 93(3):339-43. (1994) PMID 8125488 417 Davignon J, Roederer G, Montigny M, Hayden MR, Tan MH, Connelly PW, Hegele R, McPherson R, Lupien PJ, Gagné C. Comparative efficacy and safety of pravastatin, nicotinic acid and the two combined in patients with hypercholesterolemia. Am. J. Cardiol. 73(5):339-45. (1994) PMID 8109547 418 Ma Y, Ooi TC, Liu MS, Zhang H, McPherson R, Edwards AL, Forsythe IJ, Frohlich J, Brunzell JD, Hayden MR. High frequency of mutations in the human lipoprotein lipase gene in pregnancy-induced chylomicronemia: possible association with apolipoprotein E2 isoform. J. Lipid Res. 35(6):1066-75. (1994) PMID 8077845 419 Karlinsky H, Sadovnick AD, Burgess MM, Langlois S, Hayden MR, Berg JM. Issues in molecular genetic testing of individuals with suspected early-onset familial Alzheimer's disease. Alzheimer Dis Assoc Disord 8(2):116-25. (1994) PMID 8060604 420 Broholm J, Cassiman J-J, Craufurd D, Falek A, Farmer-Little C, Hayden MR, Kapp R, Krahnen K, Martinez-Descals A, Mol M, Myrianthopolous N, Petit H, Quaid K, De Simviele C, Taylor E, Tyler A, Walker R, Went L, Wexler N. Guidelines for the molecular genetics predictive test in Huntington's disease. International Huntington Association (IHA) and the World Federation of Neurology (WFN) Research Group on Huntington's Chorea. Neurology 44(8):1533-6. (1994) PMID 8058167 421 Telenius H, Kremer B, Goldberg YP, Theilmann J, Andrew SE, Zeisler J, Adam S, Greenberg C, Ives EJ, Clarke LA. Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm. Nat. Genet. 6(4):409-14. (1994) PMID 8054984 422 Gagné E, Genest J, Zhang H, Clarke LA, Hayden MR. Analysis of DNA changes in the LPL gene in patients with familial combined hyperlipidemia. Arterioscler. Thromb. 14(8):1250-7. (1994) PMID 8049185 423 Almqvist E, Andrew S, Theilmann J, Goldberg P, Zeisler J, Drugge U, Grandell U, Tapper-Persson M, Winblad B, Hayden M. Geographical distribution of haplotypes in Swedish families with Huntington's disease. Hum. Genet. 94(2):124-8. (1994) PMID 8045558 424 Hayden MR, Liu MS, Ma Y. Gene environment interaction and plasma triglyceride levels: the crucial role of lipoprotein lipase. Clin. Genet. 46(1 Spec No):15-8. (1994) PMID 7988072 425 Shinotoh H, Calne DB, Snow B, Hayward M, Kremer B, Theilmann J, Hayden MR. Normal CAG repeat length in the Huntington's disease gene in senile chorea. Neurology 44(11):2183-4. (1994) PMID 7969980 426 Nasir J, Lin B, Bucan M, Koizumi T, Nadeau JH, Hayden MR. The murine homologues of the Huntington disease gene (Hdh) and the alpha-adducin gene (Add1) map to mouse chromosome 5 within a region of conserved synteny with human chromosome 4p16.3. Genomics 22(1):198-201. (1994) PMID 7959767 427 Goldberg YP, Telenius H, Hayden MR. The molecular genetics of Huntington's disease. Curr. Opin. Neurol. 7(4):325-32. (1994) PMID 7952241 428 Benjamin CM, Adam S, Wiggins S, Theilmann JL, Copley TT, Bloch M, Squitieri F, McKellin W, Cox S, Brown SA. Proceed with care: direct predictive testing for Huntington disease. Am. J. Hum. Genet. 55(4):606-17. (1994) PMID 7942838 429 Bruin T, Tuzgöl S, Mulder WJ, van den Ende AE, Jansen H, Hayden MR, Kastelein JJ. A compound heterozygote for lipoprotein lipase deficiency, Val69-->Leu and Gly188-->Glu: correlation between in vitro LPL activity and clinical expression. J. Lipid Res. 35(3):438-45. (1994) PMID 7912254 430 Ma Y, Liu MS, Chitayat D, Bruin T, Beisiegel U, Benlian P, Foubert L, De Gennes JL, Funke H, Forsythe I. Recurrent missense mutations at the first and second base of codon Arg243 in human lipoprotein lipase in patients of different ancestries. Hum. Mutat. 3(1):52-8. (1994) PMID 7906986 431 Squitieri F, Andrew SE, Goldberg YP, Kremer B, Spence N, Zeisler J, Nichol K, Theilmann J, Greenberg J, Goto J. DNA haplotype analysis of Huntington disease reveals clues to the origins and mechanisms of CAG expansion and reasons for geographic variations of prevalence. Hum. Mol. Genet. 3(12):2103-14. (1994) PMID 7881406 432 Ma Y, Henderson HE, Liu MS, Zhang H, Forsythe IJ, Clarke-Lewis I, Hayden MR, Brunzell JD. Mutagenesis in four candidate heparin binding regions (residues 279-282, 291-304, 390-393, and 439-448) and identification of residues affecting heparin binding of human lipoprotein lipase. J. Lipid Res. 35(11):2049-59. (1994) PMID 7868983 433 Tuzgöl S, Bijvoet SM, Bruin T, Kastelein JJ, Hayden MR. Apolipoprotein CII-Padova (Tyr37-->stop) as a cause of chylomicronaemia in an Italian kindred from Siculiana. J. Med. Genet. 31(8):622-6. (1994) PMID 7815420 434 Clarke LA, Nasir J, Zhang H, McDonald H, Applegarth DA, Hayden MR, Toone J. Murine alpha-L-iduronidase: cDNA isolation and expression. Genomics 24(2):311-6. (1994) PMID 7698753 435 Clark CM, Kremer B, Hayden MR. Regional cerebral glucose metabolism in Huntington’s Disease: A statistical investigation Human Brain Mapping 2(1-2):95-102. (1994) 436 Ma Y, Liu MS, Ginzinger D, Frohlich J, Brunzell JD, Hayden MR. Gene-environment interaction in the conversion of a mild-to-severe phenotype in a patient homozygous for a Ser172-->Cys mutation in the lipoprotein lipase gene. J. Clin. Invest. 91(5):1953-8. (1993) PMID 8486765 437 Dionne C, Gagné C, Julien P, Murthy MR, Roederer G, Davignon J, Lambert M, Chitayat D, Ma R, Henderson H. Genealogy and regional distribution of lipoprotein lipase deficiency in French-Canadians of Quebec. Hum. Biol. 65(1):29-39. (1993) PMID 8436388 438 Funke H, von Eckardstein A, Pritchard PH, Hornby AE, Wiebusch H, Motti C, Hayden MR, Dachet C, Jacotot B, Gerdes U. Genetic and phenotypic heterogeneity in familial lecithin: cholesterol acyltransferase (LCAT) deficiency. Six newly identified defective alleles further contribute to the structural heterogeneity in this disease. J. Clin. Invest. 91(2):677-83. (1993) PMID 8432868 439 Adam S, Wiggins S, Whyte P, Bloch M, Shokeir MH, Soltan H, Meschino W, Summers A, Suchowersky O, Welch JP. Five year study of prenatal testing for Huntington's disease: demand, attitudes, and psychological assessment. J. Med. Genet. 30(7):549-56. (1993) PMID 8411026 440 Collins C, Duff C, Duncan AM, Planells-Cases R, Sun W, Norremolle A, Michaelis E, Montal M, Worton R, Hayden MR. Mapping of the human NMDA receptor subunit (NMDAR1) and the proposed NMDA receptor glutamate-binding subunit (NMDARA1) to chromosomes 9q34.3 and chromosome 8, respectively. Genomics 17(1):237-9. (1993) PMID 8406459 441 Andrew SE, Goldberg YP, Kremer B, Telenius H, Theilmann J, Adam S, Starr E, Squitieri F, Lin B, Kalchman MA. The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease. Nat. Genet. 4(4):398-403. (1993) PMID 8401589 442 Weber B, Riess O, Daneshvar H, Graham R, Hayden MR. (CA)n-dinucleotide repeat at the PDEB locus in 4p16.3. Hum. Mol. Genet. 2(6):827. (1993) PMID 8394765 443 Hutchinson GB, Andrew SE, McDonald H, Goldberg YP, Graham R, Rommens JM, Hayden MR. An Alu element retroposition in two families with Huntington disease defines a new active Alu subfamily. Nucleic Acids Res. 21(15):3379-83. (1993) PMID 8393987 444 Goldberg YP, Rommens JM, Andrew SE, Hutchinson GB, Lin B, Theilmann J, Graham R, Glaves ML, Starr E, McDonald H. Identification of an Alu retrotransposition event in close proximity to a strong candidate gene for Huntington's disease. Nature 362(6418):370-3. (1993) PMID 8384324 445 Hayden MR. On planting alfalfa and growing orchids: the cloning of the gene causing Huntington disease. Clin. Genet. 43(5):217-22. (1993) PMID 8375101 446 Andrew S, Theilmann J, Almqvist E, Norremolle A, Lucotte G, Anvret M, Sorensen SA, Turpin JC, Hayden MR. DNA analysis of distinct populations suggests multiple origins for the mutation causing Huntington disease. Clin. Genet. 43(6):286-94. (1993) PMID 8370147 447 Ma Y, Liu MS, Zhang H, Forsythe IJ, Brunzell JD, Hayden MR. A 4 basepair deletion in exon 4 of the human lipoprotein lipase gene results in type I hyperlipoproteinemia. Hum. Mol. Genet. 2(7):1049-50. (1993) PMID 8364543 448 Hayden MR, Kastelein JJ, Funke H, Brunzell JD, Ma Y. Phenotypic variation of mutations in the human lipoprotein-lipase gene. Biochem. Soc. Trans. 21(2):506-9. (1993) PMID 8359520 449 Goldberg YP, Andrew SE, Clarke LA, Hayden MR. A PCR method for accurate assessment of trinucleotide repeat expansion in Huntington disease. Hum. Mol. Genet. 2(6):635-6. (1993) PMID 8353482 450 Frohlich J, Brun LD, Blank D, Campeau L, Crockford P, Curnew G, Dafoe W, Davignon J, Dufour R, Emery G. Comparison of the short term efficacy and tolerability of lovastatin and simvastatin in the management of primary hypercholesterolemia. Can J Cardiol 9(5):405-12. (1993) PMID 8348392 451 Durbach N, Hayden MR. George Huntington: the man behind the eponym. J. Med. Genet. 30(5):406-9. (1993) PMID 8320704 452 Bruin T, Tuzgöl S, van Diermen DE, Hoogerbrugge-van der Linden N, Brunzell JD, Hayden MR, Kastelein JJ. Recurrent pancreatitis and chylomicronemia in an extended Dutch kindred is caused by a Gly154-->Ser substitution in lipoprotein lipase. J. Lipid Res. 34(12):2109-19. (1993) PMID 8301230 453 Telenius H, Kremer HP, Theilmann J, Andrew SE, Almqvist E, Anvret M, Greenberg C, Greenberg J, Lucotte G, Squitieri F. Molecular analysis of juvenile Huntington disease: the major influence on (CAG)n repeat length is the sex of the affected parent. Hum. Mol. Genet. 2(10):1535-40. (1993) PMID 8268906 454 Goldberg YP, Kremer B, Andrew SE, Theilmann J, Graham RK, Squitieri F, Telenius H, Adam S, Sajoo A, Starr E. Molecular analysis of new mutations for Huntington's disease: intermediate alleles and sex of origin effects. Nat. Genet. 5(2):174-9. (1993) PMID 8252043 455 Babul R, Adam S, Kremer B, Dufrasne S, Wiggins S, Huggins M, Theilmann J, Bloch M, Hayden MR. Attitudes toward direct predictive testing for the Huntington disease gene. Relevance for other adult-onset disorders. The Canadian Collaborative Group on Predictive Testing for Huntington Disease. JAMA 270(19):2321-5. (1993) PMID 8230594 456 Henderson HE, Ma Y, Liu MS, Clark-Lewis I, Maeder DL, Kastelein JJ, Brunzell JD, Hayden MR. Structure-function relationships of lipoprotein lipase: mutation analysis and mutagenesis of the loop region. J. Lipid Res. 34(9):1593-602. (1993) PMID 8228642 457 Defesche JC, Pricker KL, Hayden MR, van der Ende BE, Kastelein JJ. Familial defective apolipoprotein B-100 is clinically indistinguishable from familial hypercholesterolemia. Arch. Intern. Med. 153(20):2349-56. (1993) PMID 8215738 458 Hayden MR, Josephson R. Development of a program for identification of patients with familial hypercholesterolemia in British Columbia: a model for prevention of coronary disease. Am. J. Cardiol. 72(10):25D-29D. (1993) PMID 8213493 459 Bloch M, Adam S, Fuller A, Kremer B, Welch JP, Wiggins S, Whyte P, Huggins M, Theilmann J, Hayden MR. Diagnosis of Huntington disease: a model for the stages of psychological response based on experience of a predictive testing program. Am. J. Med. Genet. 47(3):368-74. (1993) PMID 8135283 460 Kremer B, Squitieri F, Telenius H, Andrew SE, Theilmann J, Spence N, Goldberg YP, Hayden MR. Molecular analysis of late onset Huntington's disease. J. Med. Genet. 30(12):991-5. (1993) PMID 8133510 461 Goldberg YP, Andrew SE, Theilmann J, Kremer B, Squitieri F, Telenius H, Brown JD, Hayden MR. Familial predisposition to recurrent mutations causing Huntington's disease: genetic risk to sibs of sporadic cases. J. Med. Genet. 30(12):987-90. (1993) PMID 8133509 462 . Presymptomatic testing for Huntington's disease: a world wide survey. The World Federation of Neurology Research Group on Huntington's Disease. J. Med. Genet. 30(12):1020-2. (1993) PMID 8133500 463 McGeer EG, Kremer B, Hayden MR. Monoamines and their metabolites in Huntington's disease brain: evidence for decreased catechol-O-methyltransferase activity. Biol. Psychiatry 33(7):551-3. (1993) PMID 8099814 464 Wood S, Schertzer M, Hayden M, Ma Y. Support for founder effect for two lipoprotein lipase (LPL) gene mutations in French Canadians by analysis of GT microsatellites flanking the LPL gene. Hum. Genet. 91(4):312-6. (1993) PMID 8099055 465 Zambon A, Torres A, Bijvoet S, Gagne C, Moorjani S, Lupien PJ, Hayden MR, Brunzell JD. Prevention of raised low-density lipoprotein cholesterol in a patient with familial hypercholesterolaemia and lipoprotein lipase deficiency. Lancet 341(8853):1119-21. (1993) PMID 8097805 466 Lin B, Rommens JM, Graham RK, Kalchman M, MacDonald H, Nasir J, Delaney A, Goldberg YP, Hayden MR. Differential 3' polyadenylation of the Huntington disease gene results in two mRNA species with variable tissue expression. Hum. Mol. Genet. 2(10):1541-5. (1993) PMID 7903579 467 Defesche JC, van Diermen DE, Lansberg PJ, Lamping RJ, Reymer PW, Hayden MR, Kastelein JJ. South African founder mutations in the low-density lipoprotein receptor gene causing familial hypercholesterolemia in the Dutch population. Hum. Genet. 92(6):567-70. (1993) PMID 7903269 468 Rommens JM, Lin B, Hutchinson GB, Andrew SE, Goldberg YP, Glaves ML, Graham R, Lai V, McArthur J, Nasir J. A transcription map of the region containing the Huntington disease gene. Hum. Mol. Genet. 2(7):901-7. (1993) PMID 7689900 469 Tooyama I, Kremer HP, Hayden MR, Kimura H, McGeer EG, McGeer PL. Acidic and basic fibroblast growth factor-like immunoreactivity in the striatum and midbrain in Huntington's disease. Brain Res. 610(1):1-7. (1993) PMID 7686078 470 Kassen B, Hayden MR, Busser JR, Ensworth SG, Ross JS. Hemochromatosis BC Med J 135:31-33. (1993) 471 Ma YH, Bruin T, Tuzgol S, Wilson BI, Roederer G, Liu MS, Davignon J, Kastelein JJ, Brunzell JD, Hayden MR. Two naturally occurring mutations at the first and second bases of codon aspartic acid 156 in the proposed catalytic triad of human lipoprotein lipase. In vivo evidence that aspartic acid 156 is essential for catalysis. J. Biol. Chem. 267(3):1918-23. (1992) PMID 1730727 472 Ma Y, Wilson BI, Bijvoet S, Henderson HE, Cramb E, Roederer G, Ven Murthy MR, Julien P, Bakker HD, Kastelein JJ. A missense mutation (Asp250----Asn) in exon 6 of the human lipoprotein lipase gene causes chylomicronemia in patients of different ancestries. Genomics 13(3):649-53. (1992) PMID 1639392 473 Bergeron J, Normand T, Bharucha A, Ven Murthy MR, Julien P, Gagné C, Dionne C, De Braekeleer M, Brun D, Hayden MR. Prevalence, geographical distribution and genealogical investigations of mutation 188 of lipoprotein lipase gene in the French Canadian population of Québec. Clin. Genet. 41(4):206-10. (1992) PMID 1576758 474 Bloch M, Adam S, Wiggins S, Huggins M, Hayden MR. Predictive testing for Huntington disease in Canada: the experience of those receiving an increased risk. Am. J. Med. Genet. 42(4):499-507. (1992) PMID 1535177 475 Huggins M, Bloch M, Wiggins S, Adam S, Suchowersky O, Trew M, Klimek ML, Greenberg CR, Eleff M, Thompson LP, Knight J, MacLeod P, Girard K, Theilmann J, Hedrick A, Hayden MR. Predictive testing for Huntington disease. J Med Ethics 18(1):47-8. (1992) PMID 1533425 476 Martin WR, Clark C, Ammann W, Stoessl AJ, Shtybel W, Hayden MR. Cortical glucose metabolism in Huntington's disease. Neurology 42(1):223-9. (1992) PMID 1531155 477 Dionne C, Gagné C, Julien P, Murthy MR, Lambert M, Roederer G, Davignon J, Hayden MR, Lupien PJ, de Braekeleer M. Genetic epidemiology of lipoprotein lipase deficiency in Saguenay-Lac-St-Jean (Québec, Canada). Ann. Genet. 35(2):89-92. (1992) PMID 1524414 478 Bruin T, Kastelein JJ, Van Diermen DE, Ma Y, Henderson HE, Stuyt PM, Stalenhoef AF, Sturk A, Brunzell JD, Hayden MR. A missense mutation Pro157 Arg in lipoprotein lipase (LPLNijmegen) resulting in loss of catalytic activity. Eur. J. Biochem. 208(2):267-72. (1992) PMID 1521525 479 Hayden MR, Ma Y. Molecular genetics of human lipoprotein lipase deficiency. Mol. Cell. Biochem. 113(2):171-6. (1992) PMID 1518507 480 Normand T, Bergeron J, Fernandez-Margallo T, Bharucha A, Ven Murthy MR, Julien P, Gagné C, Dionne C, De Braekeleer M, Ma R. Geographic distribution and genealogy of mutation 207 of the lipoprotein lipase gene in the French Canadian population of Québec. Hum. Genet. 89(6):671-5. (1992) PMID 1511985 481 Defesche JC, van de Ree MA, Kastelein JJ, van Diermen DE, Janssens NW, van Doormaal JJ, Hayden MR. Detection of the Pro664-Leu mutation in the low-density lipoprotein receptor and its relation to lipoprotein(a) levels in patients with familial hypercholesterolemia of Dutch ancestry from The Netherlands and Canada. Clin. Genet. 42(6):273-80. (1992) PMID 1493640 482 Wiggins S, Whyte P, Huggins M, Adam S, Theilmann J, Bloch M, Sheps SB, Schechter MT, Hayden MR. The psychological consequences of predictive testing for Huntington's disease. Canadian Collaborative Study of Predictive Testing. N. Engl. J. Med. 327(20):1401-5. (1992) PMID 1406858 483 Liu MS, Ma Y, Hayden MR, Brunzell JD. Mapping of the epitope on lipoprotein lipase recognized by a monoclonal antibody (5D2) which inhibits lipase activity. Biochim. Biophys. Acta 1128(1):113-5. (1992) PMID 1382603 484 Henderson HE, Hassan F, Berger GM, Hayden MR. The lipoprotein lipase Gly188----Glu mutation in South Africans of Indian descent: evidence suggesting common origins and an increased frequency. J. Med. Genet. 29(2):119-22. (1992) PMID 1351946 485 Andrew S, Theilmann J, Hedrick A, Mah D, Weber B, Hayden MR. Nonrandom association between Huntington disease and two loci separated by about 3 Mb on 4p16.3. Genomics 13(2):301-11. (1992) PMID 1351866 486 Weber B, Hedrick A, Andrew S, Riess O, Collins C, Kowbel D, Hayden MR. Isolation and characterization of new highly polymorphic DNA markers from the Huntington disease region. Am. J. Hum. Genet. 50(2):382-93. (1992) PMID 1346482 487 Weber B, Riess O, Wolff G, Andrew S, Collins C, Graham R, Theilmann J, Hayden MR. Delineation of a 50 kilobase DNA segment containing the recombination site in a sporadic case of Huntington's disease. Nat. Genet. 2(3):216-22. (1992) PMID 1345172 488 Kremer B, Weber B, Hayden MR. New insights into the clinical features, pathogenesis and molecular genetics of Huntington disease. Brain Pathol. 2(4):321-35. (1992) PMID 1341966 489 Riess O, Noerremoelle A, Collins C, Mah D, Weber B, Hayden MR. Exclusion of DNA changes in the beta-subunit of the c-GMP phosphodiesterase gene as the cause for Huntington's disease. Nat. Genet. 1(2):104-8. (1992) PMID 1338767 490 Riess O, Weber B, Noeremolle A, Shaikh RA, Hayden MR, Musarella MA. Linkage studies and mutation analysis of the PDEB gene in 23 families with Leber congenital amaurosis. Hum. Mutat. 1(6):478-85. (1992) PMID 1338765 491 Riess O, Weber B, Hayden MR. (CA)n-dinucleotide repeat polymorphism at the locus for the alpha2C adrenergic receptor (ADRA2C) on 4p16. Hum. Mol. Genet. 1(6):452. (1992) PMID 1338762 492 Liang XR. [Postoperative care of multiple familial colonic polyposis] 27(11):498-9. (1992) PMID 1338715 493 Riess O, Noerremoelle A, Weber B, Musarella MA, Hayden MR. The search for mutations in the gene for the beta subunit of the cGMP phosphodiesterase (PDEB) in patients with autosomal recessive retinitis pigmentosa. Am. J. Hum. Genet. 51(4):755-62. (1992) PMID 1329504 494 Collins C, Hutchinson G, Kowbel D, Riess O, Weber B, Hayden MR. The human beta-subunit of rod photoreceptor cGMP phosphodiesterase: complete retinal cDNA sequence and evidence for expression in brain. Genomics 13(3):698-704. (1992) PMID 1322354 495 Hutchinson GB, Hayden MR. The prediction of exons through an analysis of spliceable open reading frames. Nucleic Acids Res. 20(13):3453-62. (1992) PMID 1321415 496 Bijvoet SM, Hayden MR. Mismatch PCR: a rapid method to screen for the Pro207-->Leu mutation in the lipoprotein lipase (LPL) gene. Hum. Mol. Genet. 1(7):541. (1992) PMID 1307255 497 Collins C, Schappert K, Hayden MR. The genomic organization of a novel regulatory myosin light chain gene (MYL5) that maps to chromosome 4p16.3 and shows different patterns of expression between primates. Hum. Mol. Genet. 1(9):727-33. (1992) PMID 1284596 498 Goldberg YP, Lin BY, Andrew SE, Nasir J, Graham R, Glaves ML, Hutchinson G, Theilmann J, Ginzinger DG, Schappert K. Cloning and mapping of the alpha-adducin gene close to D4S95 and assessment of its relationship to Huntington disease. Hum. Mol. Genet. 1(9):669-75. (1992) PMID 1284592 499 Brunzell JD, Peterson J, Deeb SS, Santamarina-Fojo S, Julien P, Ma Y, Henderson HE, Hayden MR. Familial lipoprotein lipase deficiency Atherosclerosis IX, Proc. of the 9th Intl. Symposium on Atherosclerosis 1:271-273. (1992) 500 Ma Y, Henderson HE, Murthy V, Roederer G, Monsalve MV, Clarke LA, Normand T, Julien P, Gagné C, Lambert M. A mutation in the human lipoprotein lipase gene as the most common cause of familial chylomicronemia in French Canadians. N. Engl. J. Med. 324(25):1761-6. (1991) PMID 2038366 501 Hill JS, Hayden MR, Frohlich J, Pritchard PH. Genetic and environmental factors affecting the incidence of coronary artery disease in heterozygous familial hypercholesterolemia. Arterioscler. Thromb. 11(2):290-7. (1991) PMID 1998646 502 Weber B, Collins C, Kowbel D, Riess O, Hayden MR. Identification of multiple CpG islands and associated conserved sequences in a candidate region for the Huntington disease gene. Genomics 11(4):1113-24. (1991) PMID 1838348 503 Hayden MR. Predictive testing for Huntington disease: are we ready for widespread community implementation? Am. J. Med. Genet. 40(4):515-7. (1991) PMID 1836106 504 Greenberg LJ, Martell RW, Theilman J, Hayden MR, Joubert J. Genetic linkage between Huntington disease and the D4S10 locus in South African families: further evidence against non-allelic heterogeneity. Hum. Genet. 87(6):701-8. (1991) PMID 1834541 505 Clark CM, Ammann W, Martin WR, Ty P, Hayden MR. The FDG/PET methodology for early detection of disease onset: a statistical model. J. Cereb. Blood Flow Metab. 11(2):A96-102. (1991) PMID 1825496 506 Weber B, Allen L, Magenis RE, Goodfellow PJ, Smith L, Hayden MR. Intrachromosomal location of the telomeric repeat (TTAGGG)n. Mamm. Genome 1(4):211-6. (1991) PMID 1794050 507 Weber B, Allen L, Magenis RE, Hayden MR. A low-copy repeat located in subtelomeric regions of 14 different human chromosomal termini. Cytogenet. Cell Genet. 57(4):179-83. (1991) PMID 1743071 508 Weber B, Riess O, Hutchinson G, Collins C, Lin BY, Kowbel D, Andrew S, Schappert K, Hayden MR. Genomic organization and complete sequence of the human gene encoding the beta-subunit of the cGMP phosphodiesterase and its localisation to 4p 16.3. Nucleic Acids Res. 19(22):6263-8. (1991) PMID 1720239 509 Henderson HE, Ma Y, Hassan MF, Monsalve MV, Marais AD, Winkler F, Gubernator K, Peterson J, Brunzell JD, Hayden MR. Amino acid substitution (Ile194----Thr) in exon 5 of the lipoprotein lipase gene causes lipoprotein lipase deficiency in three unrelated probands. Support for a multicentric origin. J. Clin. Invest. 87(6):2005-11. (1991) PMID 1674945 510 Weber B, Riess O, Kreklywich CN, Wood S, Hayden MR. A polymorphic DNA marker at the D8S131 locus. Nucleic Acids Res. 19(7):1725. (1991) PMID 1674144 511 Weber B, Riess O, Hayden MR. A polymorphic DNA marker at the D10S106 locus. Nucleic Acids Res. 19(7):1725. (1991) PMID 1674143 512 Adam S, Theilmann J, Buetow K, Hedrick A, Collins C, Weber B, Huggins M, Hayden M. Linkage disequilibrium and modification of risk for Huntington disease. Am. J. Hum. Genet. 48(3):595-603. (1991) PMID 1671809 513 Eisen A, Bohlega S, Bloch M, Hayden M. Silent periods, long-latency reflexes and cortical MEPs in Huntington's disease and at-risk relatives. Electroencephalogr Clin Neurophysiol 74(6):444-9. (1990) PMID 2480224 514 Clark C, Klonoff H, Hayden M. Regional cerebral glucose metabolism in Turner syndrome. Can J Neurosci 17(2):140-4. (1990) PMID 2357649 515 Weber B, Collins C, Robbins C, Magenis RE, Delaney AD, Gray JW, Hayden MR. Characterization and organization of DNA sequences adjacent to the human telomere associated repeat (TTAGGG)n. Nucleic Acids Res. 18(11):3353-61. (1990) PMID 2356126 516 Devlin RH, Deeb S, Brunzell J, Hayden MR. Partial gene duplication involving exon-Alu interchange results in lipoprotein lipase deficiency. Am. J. Hum. Genet. 46(1):112-9. (1990) PMID 2294743 517 Peritz LN, Brunzell JD, Harvey-Clarke C, Pritchard PH, Jones BR, Hayden MR. Characterization of a lipoprotein lipase class III type defect in hypertriglyceridemic cats. Clin Invest Med 13(5):259-63. (1990) PMID 2276220 518 Bloch M, Hayden MR. DNA testing for Huntington disease results in a modification of risk and not diagnosis of disease. Eur. J. Pediatr. 149(7):513. (1990) PMID 2140774 519 Bloch M, Hayden MR. Opinion: predictive testing for Huntington disease in childhood: challenges and implications. Am. J. Hum. Genet. 46(1):1-4. (1990) PMID 2136787 520 Henderson HE, Devlin R, Peterson J, Brunzell JD, Hayden MR. Frameshift mutation in exon 3 of the lipoprotein lipase gene causes a premature stop codon and lipoprotein lipase deficiency. Mol. Biol. Med. 7(6):511-7. (1990) PMID 2077351 521 Monsalve MV, Henderson H, Roederer G, Julien P, Deeb S, Kastelein JJ, Peritz L, Devlin R, Bruin T, Murthy MR. A missense mutation at codon 188 of the human lipoprotein lipase gene is a frequent cause of lipoprotein lipase deficiency in persons of different ancestries. J. Clin. Invest. 86(3):728-34. (1990) PMID 1975597 522 Huggins M, Bloch M, Kanani S, Quarrell OW, Theilman J, Hedrick A, Dickens B, Lynch A, Hayden M. Ethical and legal dilemmas arising during predictive testing for adult-onset disease: the experience of Huntington disease. Am. J. Hum. Genet. 47(1):4-12. (1990) PMID 1971997 523 Kastelein JJ, Haines JL, Hayden MR. The gene causing familial hypoalphalipoproteinemia is not caused by a defect in the apo AI-CIII-AIV gene cluster in a Spanish family. Hum. Genet. 84(5):396-400. (1990) PMID 1969839 524 Lowry RB, Wood BJ, Cox TA, Hayden MR. Epiphyseal dysplasia, microcephaly, nystagmus, and retinitis pigmentosa. Am. J. Med. Genet. 33(3):341-5. (1989) PMID 2801768 526 Hayden MR. The genetic aspects of atherosclerosis and hyperlipidemia. CMAJ 141(2):135. (1989) PMID 2743228 527 Chitayat D, Davis EB, McGillivray BC, Hayden MR, Hall JG. Perinatal and first year follow-up of patients with Prader-Willi syndrome: normal size of hands and feet. Clin. Genet. 35(3):161-6. (1989) PMID 2706799 528 Quarrell OW, Bloch M, Hayden MR. Insurance and the presymptomatic diagnosis of delayed-onset disease. JAMA 262(17):2384-5. (1989) PMID 2619811 529 Conneally PM, Haines JL, Tanzi RE, Wexler NS, Penchaszadeh GK, Harper PS, Folstein SE, Cassiman JJ, Myers RH, Young AB. Huntington disease: no evidence for locus heterogeneity. Genomics 5(2):304-8. (1989) PMID 2571579 530 Gessler M, Thomas GH, Couillin P, Junien C, McGillivray BC, Hayden M, Jaschek G, Bruns GA. A deletion map of the WAGR region on chromosome 11. Am. J. Hum. Genet. 44(4):486-95. (1989) PMID 2539014 531 Langlois S, Deeb S, Brunzell JD, Kastelein JJ, Hayden MR. A major insertion accounts for a significant proportion of mutations underlying human lipoprotein lipase deficiency. Proc. Natl. Acad. Sci. U.S.A. 86(3):948-52. (1989) PMID 2536938 532 Theilmann J, Kanani S, Shiang R, Robbins C, Quarrell O, Huggins M, Hedrick A, Weber B, Collins C, Wasmuth JJ. Non-random association between alleles detected at D4S95 and D4S98 and the Huntington's disease gene. J. Med. Genet. 26(11):676-81. (1989) PMID 2531224 533 Theilmann JL, Robbins CA, Hayden MR. Methylation at the D4S95 locus and predictive testing. Am. J. Hum. Genet. 45(3):477-9. (1989) PMID 2528290 534 Fahy M, Robbins C, Bloch M, Turnell RW, Hayden MR. Different options for prenatal testing for Huntington's disease using DNA probes. J. Med. Genet. 26(6):353-7. (1989) PMID 2525622 535 Bloch M, Fahy M, Fox S, Hayden MR. Predictive testing for Huntington disease: II. Demographic characteristics, life-style patterns, attitudes, and psychosocial assessments of the first fifty-one test candidates. Am. J. Med. Genet. 32(2):217-24. (1989) PMID 2522735 536 Fox S, Bloch M, Fahy M, Hayden MR. Predictive testing for Huntington disease: I. Description of a pilot project in British Columbia. Am. J. Med. Genet. 32(2):211-6. (1989) PMID 2522734 537 Robbins C, Theilmann J, Youngman S, Haines J, Altherr MJ, Harper PS, Payne C, Junker A, Wasmuth J, Hayden MR. Evidence from family studies that the gene causing Huntington disease is telomeric to D4S95 and D4S90. Am. J. Hum. Genet. 44(3):422-5. (1989) PMID 2521771 538 Chitayat D, Lao A, Wilson RD, Fagerstrom C, Hayden M. Prenatal diagnosis of asplenia/polysplenia syndrome. Am. J. Obstet. Gynecol. 158(5):1085-7. (1988) PMID 3369489 539 Bamforth S, Hayden MR. Pulmonary emphysema in neonate with the Marfan syndrome. Pediatr Radiol 18(1):88. (1988) PMID 3340437 540 Makrides SC, Ruiz-Opazo N, Hayden M, Nussbaum AL, Breslow JL, Zannis VI. Sequence and expression of Tangier apoA-I gene. Eur. J. Biochem. 173(2):465-71. (1988) PMID 3129297 541 Rabkin SW, Hayden M, Frohlich J. Comparison of gemfibrozil and clofibrate on serum lipids in familial combined hyperlipidemia. A randomized placebo-controlled, double-blind, crossover clinical trial. Atherosclerosis 73(2-3):233-40. (1988) PMID 3056431 542 Hayden MR, Robbins C, Allard D, Haines J, Fox S, Wasmuth J, Fahy M, Bloch M. Improved predictive testing for Huntington disease by using three linked DNA markers. Am. J. Hum. Genet. 43(5):689-94. (1988) PMID 2973229 543 Lam RW, Bloch M, Jones BD, Marcus AM, Fox S, Amman W, Hayden MR. Psychiatric morbidity associated with early clinical diagnosis of Huntington disease in a predictive testing program. J Clin Psychiatry 49(11):444-7. (1988) PMID 2972696 544 Hayden MR, Bloch M, Fahy M. Predictive testing for Huntington's disease using linked DNA markers. N. Engl. J. Med. 319(9):583-4. (1988) PMID 2970011 545 Jason GW, Pajurkova EM, Suchowersky O, Hewitt J, Hilbert C, Reed J, Hayden MR. Presymptomatic neuropsychological impairment in Huntington's disease. Arch. Neurol. 45(7):769-73. (1988) PMID 2968784 546 Wasmuth JJ, Hewitt J, Smith B, Allard D, Haines JL, Skarecky D, Partlow E, Hayden MR. A highly polymorphic locus very tightly linked to the Huntington's disease gene. Nature 332(6166):734-6. (1988) PMID 2895895 547 Hayden MR, Hewitt J, Wasmuth JJ, Kastelein JJ, Langlois S, Conneally M, Haines J, Smith B, Hilbert C, Allard D. A polymorphic DNA marker that represents a conserved expressed sequence in the region of the Huntington disease gene. Am. J. Hum. Genet. 42(1):125-31. (1988) PMID 2892395 548 Langlois S, Kastelein JJ, Hayden MR. Characterization of six partial deletions in the low-density-lipoprotein (LDL) receptor gene causing familial hypercholesterolemia (FH). Am. J. Hum. Genet. 43(1):60-8. (1988) PMID 2837085 549 Frohlich J, Fong B, Julien P, Despres JP, Angel A, Hayden M, McLeod R, Chow C, Davison RH, Pritchard H. Interaction of high density lipoprotein with adipocytes in a new patient with Tangier disease. Clin Invest Med 10(5):377-82. (1987) PMID 3677505 550 Frohlich J, Hoag G, McLeod R, Hayden M, Godin DV, Wadsworth LD, Critchley JD, Pritchard PH. Hypoalphalipoproteinemia resembling fish eye disease. Acta Med Scand 221(3):291-8. (1987) PMID 3591467 551 Hayden MR, Kastelein JJ, Langlois S. Insufficient evidence to invoke defects in or around the A-I gene as the cause for familial hypoalphalipoproteinemia. Atherosclerosis 67(2-3):271-2. (1987) PMID 3118895 552 Swart AL, Hesseling PB, Hayden MR, Louw M, Herbert JS. Gaucher's disease in the Cape coloured population of the RSA, including a family with 5 affected siblings. S. Afr. Med. J. 71(2):97-9. (1987) PMID 3101207 553 Martin WR, Hayden MR. Cerebral glucose and dopa metabolism in movement disorders. Can J Neurosci 14(3 Suppl):448-51. (1987) PMID 2960430 554 Bloch M, Hayden MR. Preclinical testing in Huntington disease. Am. J. Med. Genet. 27(3):733-4. (1987) PMID 2957915 555 Hayden MR, Hewitt J, Stoessl AJ, Clark C, Ammann W, Martin WR. The combined use of positron emission tomography and DNA polymorphisms for preclinical detection of Huntington's disease. Neurology 37(9):1441-7. (1987) PMID 2957611 556 Clark C, Hayden M, Hollenberg S, Li D, Stoessl AJ. Controlling for cerebral atrophy in positron emission tomography data. J. Cereb. Blood Flow Metab. 7(4):510-2. (1987) PMID 2956273 557 Hayden MR, Goldblatt J, Wallis G, Winship IM, Beighton P. Molecular genetics and Huntington's disease. The South African situation. S. Afr. Med. J. 71(11):683-6. (1987) PMID 2954228 558 Talmud PJ, Barni N, Kessling AM, Carlsson P, Darnfors C, Bjursell G, Galton D, Wynn V, Kirk H, Hayden MR. Apolipoprotein B gene variants are involved in the determination of serum cholesterol levels: a study in normo- and hyperlipidaemic individuals. Atherosclerosis 67(1):81-9. (1987) PMID 2890359 559 MacDonald ME, Anderson MA, Gilliam TC, Tranejaerg L, Carpenter NJ, Magenis E, Hayden MR, Healey ST, Bonner TI, Gusella JF. A somatic cell hybrid panel for localizing DNA segments near the Huntington's disease gene. Genomics 1(1):29-34. (1987) PMID 2889660 560 Hayden MR, Hewitt J, Maresca A, Langlois S. A polymorphic DNA probe located to human chromosome 4p16 (D4S62). Nucleic Acids Res. 15(9):3938. (1987) PMID 2884639 561 Hayden MR, Hewitt J, Kastelein JJ, Langlois S, Wilson RD, Fox S, Hilbert C, Bloch M. First-trimester prenatal diagnosis for Huntington's disease with DNA probes. Lancet 1(8545):1284-5. (1987) PMID 2884412 562 Hayden MR, Kirk H, Clark C, Frohlich J, Rabkin S, McLeod R, Hewitt J. DNA polymorphisms in and around the Apo-A1-CIII genes and genetic hyperlipidemias. Am. J. Hum. Genet. 40(5):421-30. (1987) PMID 2883893 563 Holbrook KA, Dale BA, Witt DR, Hayden MR, Toriello HV. Arrested epidermal morphogenesis in three newborn infants with a fatal genetic disorder (restrictive dermopathy). J. Invest. Dermatol. 88(3):330-9. (1987) PMID 2434579 564 Hayden MR, Hewitt J, Martin WRW, Clark C, Ammann W. Studies in persons at risk for Huntington’s disease N Engl J Med 317:382-383. (1987) 565 Suchowersky O, Hayden MR, Martin WR, Stoessl AJ, Hildebrand AM, Pate BD. Cerebral metabolism of glucose in benign hereditary chorea. Mov. Disord. 1(1):33-44. (1986) PMID 2973557 566 Clark CM, Hayden MR, Stoessl AJ, Martin WR. Regression model for predicting dissociations of regional cerebral glucose metabolism in individuals at risk for Huntington's disease. J. Cereb. Blood Flow Metab. 6(6):756-62. (1986) PMID 2947906 567 Wang HS, Greenberg CR, Hewitt J, Kalousek D, Hayden MR. Subregional assignment of the linked marker G8 (D4S10) for Huntington disease to chromosome 4p16.1-16.3. Am. J. Hum. Genet. 39(3):392-6. (1986) PMID 2945429 568 Bird TD, Hewitt J, Conneally PM, Hayden MR. Linkage of the G8 marker on chromosome 4 to Huntington's disease in a large American black family. N. Engl. J. Med. 315(18):1165-6. (1986) PMID 2945112 569 Froster-Iskenius UG, Hayden MR, Wang HS, Kalousek DK, Horsman D, Pfeiffer RA, Schottky A, Schwinger E. A family with Huntington disease and reciprocal translocation 4;5. Am. J. Hum. Genet. 38(5):759-67. (1986) PMID 2940859 570 Hayden MR, Martin WR, Stoessl AJ, Clark C, Hollenberg S, Adam MJ, Ammann W, Harrop R, Rogers J, Ruth T. Positron emission tomography in the early diagnosis of Huntington's disease. Neurology 36(7):888-94. (1986) PMID 2940474 571 Witt DR, Hayden MR, Holbrook KA, Dale BA, Baldwin VJ, Taylor GP. Restrictive dermopathy: a newly recognized autosomal recessive skin dysplasia. Am. J. Med. Genet. 24(4):631-48. (1986) PMID 2426945 572 Hayden MR, Nichols JL. Molecular genetic approaches to the study of the nervous system. Dev. Neurosci. 6(4-5):189-214. (1985) PMID 6083860 573 Pritchard PH, Bergseth M, McLeod R, Hayden MR, Frohlich J. Urinary proteins in a patient with Tangier disease. Clin. Biochem. 18(2):98-101. (1985) PMID 3926343 574 Wilson RD, Hayden MR. Bilateral renal agenesis in twins. Am. J. Med. Genet. 21(1):147-52, 167-9. (1985) PMID 3890542 575 Gusella JF, Tanzi RE, Bader PI, Phelan MC, Stevenson R, Hayden MR, Hofman KJ, Faryniarz AG, Gibbons K. Deletion of Huntington's disease-linked G8 (D4S10) locus in Wolf-Hirschhorn syndrome. Nature 318(6041):75-8. (1985) PMID 2997623 576 Hayden MR, Soles JA, Ward RH. Age of onset in siblings of persons with juvenile Huntington disease. Clin. Genet. 28(2):100-5. (1985) PMID 2931216 577 Siegfried E, Hayden MR, Freeman HJ. Hemochromatosis BC Med J 27:556-558. (1985) 578 Suchowersky O, Hayden M. Down's syndrome and Alzheimer's disease. Ann. Neurol. 16(2):263. (1984) PMID 6236743 579 Hayden MR, Beighton P. Genetic aspects of Huntington's chorea: results of a national survey. Am. J. Med. Genet. 11(2):135-41. (1982) PMID 6461251 580 Hayden MR, MacGregor JM, Saffer DS, Beighton PH. The high frequency of juvenile Huntington's chorea in South Africa. J. Med. Genet. 19(2):94-7. (1982) PMID 6210776 581 Hayden MR, Berkowicz AL, Beighton PH, Yiptong C. Huntington's chorea on the island of Mauritius. S. Afr. Med. J. 60(26):1001-2. (1981) PMID 6460324 583 Hayden MR, Ehrlich R, Parker H, Ferera SJ. Social perspectives in Huntington's chorea. S. Afr. Med. J. 58(5):201-3. (1980) PMID 6447366 584 Hayden MR, Hopkins HC, Macrea M, Beighton PH. The origin of Huntington's chorea in the Afrikaner population of South Africa. S. Afr. Med. J. 58(5):197-200. (1980) PMID 6447365 585 Hayden MR, MacGregor JM, Beighton PH. The prevalence of Huntington's chorea in South Africa. S. Afr. Med. J. 58(5):193-6. (1980) PMID 6447364 586 Rosman MS, Lubbe WF, Hayden M, Basson N, Uys CJ. Q fever endocarditis: a report of 2 cases. S. Afr. Med. J. 53(8):296-300. (1978) PMID 653535 587 Hayden MR, Nelson MM. The fetal alcohol syndrome. S. Afr. Med. J. 54(14):571-4. (1978) PMID 569911 588 Hayden M. The fetal hydantoin syndrome. A case report. S. Afr. Med. J. 53(4):145-6. (1978) PMID 565950 589 Tonkova-Iampol'skaia RV, Chertok TIa. [Achievements and prospects in the field of upbringing and development of young children]. Vopr Okhr Materin Det 22(7):3-5. (1977) PMID 906375 590 Hayden MR, Beighton P. Huntington's chorea in the Cape coloured community of South Africa. S. Afr. Med. J. 52(22):886-8. (1977) PMID 146919 591 Hayden MR, Vinik AI, Paul M, Beighton P. Impaired prolactin release in Huntington's chorea. Evidence for dopaminergic excess. Lancet 2(8035):423-6. (1977) PMID 70642 Invited Submissions, Books, Chapters, Letters, and Editorials1 Editorial Levine MS, Cepeda C, André VM. Location, location, location: contrasting roles of synaptic and extrasynaptic NMDA receptors in Huntington's disease. Neuron 65(2):145-7. (2010) PMID 20152121 2 Chapter Orban P, Devon RS, Hayden MR, Leavitt BR. Chapter 15 Juvenile amyotrophic lateral sclerosis. 82:301-12. (2007) PMID 18808900
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