Harry T. Orr - Publications

Affiliations: 
Neuroscience University of Minnesota, Twin Cities, Minneapolis, MN 
Area:
Neuroscience Biology, Cell Biology

213 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any innacuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2018 Friedrich J, Kordasiewicz HB, O'Callaghan B, Handler HP, Wagener C, Duvick L, Swayze EE, Rainwater O, Hofstra B, Benneyworth M, Nichols-Meade T, Yang P, Chen Z, Ortiz JP, Clark HB, ... ... Orr HT, et al. Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles. Jci Insight. 3. PMID 30385727 DOI: 10.1172/jci.insight.123193  1
2018 Bondar VV, Adamski CJ, Onur TS, Tan Q, Wang L, Diaz-Garcia J, Park J, Orr HT, Botas J, Zoghbi HY. PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in Spinocerebellar ataxia type 1. Human Molecular Genetics. PMID 29860311 DOI: 10.1093/hmg/ddy200  1
2018 Pérez Ortiz JM, Mollema N, Toker N, Adamski CJ, O'Callaghan B, Duvick L, Friedrich J, Walters MA, Strasser J, Hawkinson JE, Zoghbi HY, Henzler C, Orr HT, Lagalwar S. Reduction of protein kinase A-mediated phosphorylation of ATXN1-S776 in Purkinje cells delays onset of Ataxia in a SCA1 mouse model. Neurobiology of Disease. PMID 29758256 DOI: 10.1016/j.nbd.2018.05.002  1
2018 Rousseaux MWC, Tschumperlin T, Lu HC, Lackey EP, Bondar VV, Wan YW, Tan Q, Adamski CJ, Friedrich J, Twaroski K, Chen W, Tolar J, Henzler C, Sharma A, Bajić A, ... ... Orr HT, et al. ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism. Neuron. PMID 29526553 DOI: 10.1016/j.neuron.2018.02.013  1
2018 Orengo JP, van der Heijden ME, Hao S, Tang J, Orr HT, Zoghbi HY. Motor neuron degeneration correlates with respiratory dysfunction in SCA1. Disease Models & Mechanisms. PMID 29419414 DOI: 10.1242/dmm.032623  1
2017 Paulson HL, Shakkottai VG, Clark HB, Orr HT. Polyglutamine spinocerebellar ataxias - from genes to potential treatments. Nature Reviews. Neuroscience. PMID 28855740 DOI: 10.1038/nrn.2017.92  1
2017 Lu HC, Tan Q, Rousseaux MW, Wang W, Kim JY, Richman R, Wan YW, Yeh SY, Patel JM, Liu X, Lin T, Lee Y, Fryer JD, Han J, Chahrour M, ... ... Orr HT, et al. Disruption of the ATXN1-CIC complex causes a spectrum of neurobehavioral phenotypes in mice and humans. Nature Genetics. PMID 28288114 DOI: 10.1038/ng.3808  1
2016 Tan Q, Yalamanchili HK, Park J, De Maio A, Lu HC, Wan YW, White JJ, Bondar VV, Sayegh LS, Liu X, Gao Y, Sillitoe RV, Orr HT, Liu Z, Zoghbi HY. Extensive cryptic splicing upon loss of RBM17 and TDP43 in neurodegeneration models. Human Molecular Genetics. PMID 28007900 DOI: 10.1093/hmg/ddw337  1
2016 Ingram M, Wozniak EA, Duvick L, Yang R, Bergmann P, Carson R, O'Callaghan B, Zoghbi HY, Henzler C, Orr HT. Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways. Neuron. PMID 26948890 DOI: 10.1016/j.neuron.2016.02.011  1
2015 Lasagna-Reeves CA, Rousseaux MW, Guerrero-Munoz MJ, Vilanova-Velez L, Park J, See L, Jafar-Nejad P, Richman R, Orr HT, Kayed R, Zoghbi HY. Ataxin-1 oligomers induce local spread of pathology and decreasing them by passive immunization slows Spinocerebellar ataxia type 1 phenotypes. Elife. 4. PMID 26673892 DOI: 10.7554/eLife.10891  1
2015 Dell'Orco JM, Wasserman AH, Chopra R, Ingram MA, Hu YS, Singh V, Wulff H, Opal P, Orr HT, Shakkottai VG. Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 35: 11292-307. PMID 26269637 DOI: 10.1523/JNEUROSCI.1357-15.2015  1
2015 Lasagna-Reeves CA, Rousseaux MW, Guerrero-Muñoz MJ, Park J, Jafar-Nejad P, Richman R, Lu N, Sengupta U, Litvinchuk A, Orr HT, Kayed R, Zoghbi HY. A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1. Elife. 4. PMID 25988806 DOI: 10.7554/eLife.07558  1
2015 Gennarino VA, Singh RK, White JJ, De Maio A, Han K, Kim JY, Jafar-Nejad P, di Ronza A, Kang H, Sayegh LS, Cooper TA, Orr HT, Sillitoe RV, Zoghbi HY. Pumilio1 haploinsufficiency leads to SCA1-like neurodegeneration by increasing wild-type Ataxin1 levels. Cell. 160: 1087-98. PMID 25768905 DOI: 10.1016/j.cell.2015.02.012  1
2015 Öz G, Kittelson E, Demirgöz D, Rainwater O, Eberly LE, Orr HT, Clark HB. Assessing recovery from neurodegeneration in spinocerebellar ataxia 1: Comparison of in vivo magnetic resonance spectroscopy with motor testing, gene expression and histology. Neurobiology of Disease. 74: 158-66. PMID 25446943 DOI: 10.1016/j.nbd.2014.11.011  1
2014 Sieber BA, Landis S, Koroshetz W, Bateman R, Siderowf A, Galpern WR, Dunlop J, Finkbeiner S, Sutherland M, Wang H, Lee VM, Orr HT, Gwinn K, Ludwig K, Taylor A, et al. Prioritized research recommendations from the National Institute of Neurological Disorders and Stroke Parkinson's Disease 2014 conference. Annals of Neurology. 76: 469-72. PMID 25164235 DOI: 10.1002/ana.24261  1
2014 Opal P, Orr HT. Animal Models of Spinocerebellar Ataxia Type 1 Movement Disorders: Genetics and Models: Second Edition. 979-990. DOI: 10.1016/B978-0-12-405195-9.00063-9  1
2013 Lagalwar S, Orr HT. Regulation of ataxin-1 phosphorylation and its impact on biology. Methods in Molecular Biology (Clifton, N.J.). 1010: 201-9. PMID 23754227 DOI: 10.1007/978-1-62703-411-1_13  1
2013 Ju H, Kokubu H, Todd TW, Kahle JJ, Kim S, Richman R, Chirala K, Orr HT, Zoghbi HY, Lim J. Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 33: 9328-36. PMID 23719801 DOI: 10.1523/JNEUROSCI.3465-12.2013  1
2013 Park J, Al-Ramahi I, Tan Q, Mollema N, Diaz-Garcia JR, Gallego-Flores T, Lu HC, Lagalwar S, Duvick L, Kang H, Lee Y, Jafar-Nejad P, Sayegh LS, Richman R, Liu X, ... ... Orr HT, et al. RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1. Nature. 498: 325-31. PMID 23719381 DOI: 10.1038/nature12204  1
2013 Orr HT. Toxic RNA as a driver of disease in a common form of ALS and dementia. Proceedings of the National Academy of Sciences of the United States of America. 110: 7533-4. PMID 23630297 DOI: 10.1073/pnas.1305239110  1
2013 Ebner BA, Ingram MA, Barnes JA, Duvick LA, Frisch JL, Clark HB, Zoghbi HY, Ebner TJ, Orr HT. Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 33: 5806-20. PMID 23536093 DOI: 10.1523/JNEUROSCI.6311-11.2013  1
2013 Nelson DL, Orr HT, Warren ST. The unstable repeats--three evolving faces of neurological disease. Neuron. 77: 825-43. PMID 23473314 DOI: 10.1016/j.neuron.2013.02.022  1
2013 Mollema NJ, Orr HT. One family's search to explain a fatal neurological disorder American Scientist. 101: 442-449.  1
2012 Orr HT. SCA1-phosphorylation, a regulator of Ataxin-1 function and pathogenesis. Progress in Neurobiology. 99: 179-85. PMID 22531670 DOI: 10.1016/j.pneurobio.2012.04.003  1
2012 Orr HT. Cell biology of spinocerebellar ataxia. The Journal of Cell Biology. 197: 167-77. PMID 22508507 DOI: 10.1083/jcb.201105092  1
2012 Orr HT. Polyglutamine neurodegeneration: expanded glutamines enhance native functions. Current Opinion in Genetics & Development. 22: 251-5. PMID 22284692 DOI: 10.1016/j.gde.2012.01.001  1
2012 Ingram MA, Orr HT, Clark HB. Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias. Brain Research Bulletin. 88: 33-42. PMID 21810454 DOI: 10.1016/j.brainresbull.2011.07.016  1
2011 Fryer JD, Yu P, Kang H, Mandel-Brehm C, Carter AN, Crespo-Barreto J, Gao Y, Flora A, Shaw C, Orr HT, Zoghbi HY. Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua. Science (New York, N.Y.). 334: 690-3. PMID 22053053 DOI: 10.1126/science.1212673  1
2011 Orr HT. FTD and ALS: genetic ties that bind. Neuron. 72: 189-90. PMID 22017980 DOI: 10.1016/j.neuron.2011.10.001  1
2011 Lee Y, Fryer JD, Kang H, Crespo-Barreto J, Bowman AB, Gao Y, Kahle JJ, Hong JS, Kheradmand F, Orr HT, Finegold MJ, Zoghbi HY. ATXN1 protein family and CIC regulate extracellular matrix remodeling and lung alveolarization. Developmental Cell. 21: 746-57. PMID 22014525 DOI: 10.1016/j.devcel.2011.08.017  1
2011 Oz G, Vollmers ML, Nelson CD, Shanley R, Eberly LE, Orr HT, Clark HB. In vivo monitoring of recovery from neurodegeneration in conditional transgenic SCA1 mice. Experimental Neurology. 232: 290-8. PMID 21963649 DOI: 10.1016/j.expneurol.2011.09.021  1
2011 Barnes JA, Ebner BA, Duvick LA, Gao W, Chen G, Orr HT, Ebner TJ. Abnormalities in the climbing fiber-Purkinje cell circuitry contribute to neuronal dysfunction in ATXN1[82Q] mice. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 31: 12778-89. PMID 21900557 DOI: 10.1523/JNEUROSCI.2579-11.2011  1
2011 Hourez R, Servais L, Orduz D, Gall D, Millard I, de Kerchove d'Exaerde A, Cheron G, Orr HT, Pandolfo M, Schiffmann SN. Aminopyridines correct early dysfunction and delay neurodegeneration in a mouse model of spinocerebellar ataxia type 1. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 31: 11795-807. PMID 21849540 DOI: 10.1523/JNEUROSCI.0905-11.2011  1
2011 Lai S, O'Callaghan B, Zoghbi HY, Orr HT. 14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus. The Journal of Biological Chemistry. 286: 34606-16. PMID 21835928 DOI: 10.1074/jbc.M111.238527  1
2011 Orr HT. Are polyglutamine diseases expanding? Neuron. 70: 377-8. PMID 21555065 DOI: 10.1016/j.neuron.2011.04.015  1
2011 Gehrking KM, Andresen JM, Duvick L, Lough J, Zoghbi HY, Orr HT. Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model. Human Molecular Genetics. 20: 2204-12. PMID 21427130 DOI: 10.1093/hmg/ddr108  1
2011 Jafar-Nejad P, Ward CS, Richman R, Orr HT, Zoghbi HY. Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration. Proceedings of the National Academy of Sciences of the United States of America. 108: 2142-7. PMID 21245341 DOI: 10.1073/pnas.1018748108  1
2010 Duvick L, Barnes J, Ebner B, Agrawal S, Andresen M, Lim J, Giesler GJ, Zoghbi HY, Orr HT. SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776. Neuron. 67: 929-35. PMID 20869591 DOI: 10.1016/j.neuron.2010.08.022  1
2010 Crespo-Barreto J, Fryer JD, Shaw CA, Orr HT, Zoghbi HY. Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis. Plos Genetics. 6: e1001021. PMID 20628574 DOI: 10.1371/journal.pgen.1001021  1
2010 Orr HT. Nuclear ataxias. Cold Spring Harbor Perspectives in Biology. 2: a000786. PMID 20452956 DOI: 10.1101/cshperspect.a000786  1
2010 Oz G, Nelson CD, Koski DM, Henry PG, Marjanska M, Deelchand DK, Shanley R, Eberly LE, Orr HT, Clark HB. Noninvasive detection of presymptomatic and progressive neurodegeneration in a mouse model of spinocerebellar ataxia type 1. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 30: 3831-8. PMID 20220018 DOI: 10.1523/JNEUROSCI.5612-09.2010  1
2009 Jorgensen ND, Andresen JM, Lagalwar S, Armstrong B, Stevens S, Byam CE, Duvick LA, Lai S, Jafar-Nejad P, Zoghbi HY, Clark HB, Orr HT. Phosphorylation of ATXN1 at Ser776 in the cerebellum. Journal of Neurochemistry. 110: 675-86. PMID 19500214 DOI: 10.1111/j.1471-4159.2009.06164.x  1
2009 Carlson KM, Andresen JM, Orr HT. Emerging pathogenic pathways in the spinocerebellar ataxias. Current Opinion in Genetics & Development. 19: 247-53. PMID 19345087 DOI: 10.1016/j.gde.2009.02.009  1
2009 Carlson KM, Melcher L, Lai S, Zoghbi HY, Clark HB, Orr HT. Characterization of the zebrafish atxn1/axh gene family. Journal of Neurogenetics. 23: 313-23. PMID 19085187 DOI: 10.1080/01677060802399976  1
2009 Zoghbi HY, Orr HT. Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1. The Journal of Biological Chemistry. 284: 7425-9. PMID 18957430 DOI: 10.1074/jbc.R800041200  1
2009 Orr HT. Unstable nucleotide repeat minireview series: a molecular biography of unstable repeat disorders. The Journal of Biological Chemistry. 284: 7405. PMID 18957428 DOI: 10.1074/jbc.R800067200  1
2008 Gao Y, Zu T, Low WC, Orr HT, McIvor RS. Antisense RNA sequences modulating the ataxin-1 message: molecular model of gene therapy for spinocerebellar ataxia type 1, a dominant-acting unstable trinucleotide repeat disease. Cell Transplantation. 17: 723-34. PMID 19044200 DOI: 10.3727/096368908786516729  1
2008 Lee Y, Samaco RC, Gatchel JR, Thaller C, Orr HT, Zoghbi HY. miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis. Nature Neuroscience. 11: 1137-9. PMID 18758459 DOI: 10.1038/nn.2183  1
2008 Lim J, Crespo-Barreto J, Jafar-Nejad P, Bowman AB, Richman R, Hill DE, Orr HT, Zoghbi HY. Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature. 452: 713-8. PMID 18337722 DOI: 10.1038/nature06731  1
2008 Gatchel JR, Watase K, Thaller C, Carson JP, Jafar-Nejad P, Shaw C, Zu T, Orr HT, Zoghbi HY. The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7. Proceedings of the National Academy of Sciences of the United States of America. 105: 1291-6. PMID 18216249 DOI: 10.1073/pnas.0711257105  1
2007 Swanson MS, Orr HT. Fragile X tremor/ataxia syndrome: blame the messenger! Neuron. 55: 535-7. PMID 17698005 DOI: 10.1016/j.neuron.2007.07.032  1
2007 Jorgensen ND, Andresen JM, Pitt JE, Swenson MA, Zoghbi HY, Orr HT. Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin-1. Journal of Neurochemistry. 102: 2040-8. PMID 17540008 DOI: 10.1111/j.1471-4159.2007.04678.x  1
2007 Watase K, Gatchel JR, Sun Y, Emamian E, Atkinson R, Richman R, Mizusawa H, Orr HT, Shaw C, Zoghbi HY. Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model. Plos Medicine. 4: e182. PMID 17535104 DOI: 10.1371/journal.pmed.0040182  1
2007 Orr HT, Zoghbi HY. Trinucleotide repeat disorders. Annual Review of Neuroscience. 30: 575-621. PMID 17417937 DOI: 10.1146/annurev.neuro.29.051605.113042  1
2007 Bowman AB, Lam YC, Jafar-Nejad P, Chen HK, Richman R, Samaco RC, Fryer JD, Kahle JJ, Orr HT, Zoghbi HY. Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes. Nature Genetics. 39: 373-9. PMID 17322884 DOI: 10.1038/ng1977  1
2006 Lam YC, Bowman AB, Jafar-Nejad P, Lim J, Richman R, Fryer JD, Hyun ED, Duvick LA, Orr HT, Botas J, Zoghbi HY. ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell. 127: 1335-47. PMID 17190598 DOI: 10.1016/j.cell.2006.11.038  1
2006 Serra HG, Duvick L, Zu T, Carlson K, Stevens S, Jorgensen N, Lysholm A, Burright E, Zoghbi HY, Clark HB, Andresen JM, Orr HT. RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice. Cell. 127: 697-708. PMID 17110330 DOI: 10.1016/j.cell.2006.09.036  1
2006 He Y, Zu T, Benzow KA, Orr HT, Clark HB, Koob MD. Targeted deletion of a single Sca8 ataxia locus allele in mice causes abnormal gait, progressive loss of motor coordination, and Purkinje cell dendritic deficits. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 26: 9975-82. PMID 17005861 DOI: 10.1523/JNEUROSCI.2595-06.2006  1
2006 Riley BE, Orr HT. Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle. Genes & Development. 20: 2183-92. PMID 16912271 DOI: 10.1101/gad.1436506  1
2006 Ellerby LM, Orr HT. Neurodegenerative disease: cut to the chase. Nature. 442: 641-2. PMID 16900190 DOI: 10.1038/442641a  1
2006 Carlson KM, Orr HT. Phosphorylation Of Ataxin-1: A link between basic research and clinical application in spinocerebellar ataxia type 1 Genetic Instabilities and Neurological Diseases, Second Edition. 339-349. DOI: 10.1016/B978-012369462-1/50023-5  1
2005 Tsuda H, Jafar-Nejad H, Patel AJ, Sun Y, Chen HK, Rose MF, Venken KJ, Botas J, Orr HT, Bellen HJ, Zoghbi HY. The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Cell. 122: 633-44. PMID 16122429 DOI: 10.1016/j.cell.2005.06.012  1
2005 Vierra-Green CA, Orr HT, Zoghbi HY, Ferrington DA. Identification of a novel phosphorylation site in ataxin-1. Biochimica Et Biophysica Acta. 1744: 11-8. PMID 15878393 DOI: 10.1016/j.bbamcr.2004.10.012  1
2005 Riley BE, Zoghbi HY, Orr HT. SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal. The Journal of Biological Chemistry. 280: 21942-8. PMID 15824120 DOI: 10.1074/jbc.M501677200  1
2005 Kaytor MD, Byam CE, Tousey SK, Stevens SD, Zoghbi HY, Orr HT. A cell-based screen for modulators of ataxin-1 phosphorylation. Human Molecular Genetics. 14: 1095-105. PMID 15757972 DOI: 10.1093/hmg/ddi122  1
2005 Irwin S, Vandelft M, Pinchev D, Howell JL, Graczyk J, Orr HT, Truant R. RNA association and nucleocytoplasmic shuttling by ataxin-1. Journal of Cell Science. 118: 233-42. PMID 15615787 DOI: 10.1242/jcs.01611  1
2005 Kaytor MD, Orr HT. Animal models of spinocerebellar ataxia sype 1 (SCA1) Movement Disorders. 623-630. DOI: 10.1016/B978-012088382-0/50054-2  1
2004 Orr HT. Neurodegenerative disease: neuron protection agency. Nature. 431: 747-8. PMID 15483586 DOI: 10.1038/431747a  1
2004 Zu T, Duvick LA, Kaytor MD, Berlinger MS, Zoghbi HY, Clark HB, Orr HT. Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 24: 8853-61. PMID 15470152 DOI: 10.1523/JNEUROSCI.2978-04.2004  1
2004 Serra HG, Byam CE, Lande JD, Tousey SK, Zoghbi HY, Orr HT. Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice. Human Molecular Genetics. 13: 2535-43. PMID 15317756 DOI: 10.1093/hmg/ddh268  1
2004 Riley BE, Xu Y, Zoghbi HY, Orr HT. The effects of the polyglutamine repeat protein ataxin-1 on the UbL-UBA protein A1Up. The Journal of Biological Chemistry. 279: 42290-301. PMID 15280365 DOI: 10.1074/jbc.M406284200  1
2004 Xia H, Mao Q, Eliason SL, Harper SQ, Martins IH, Orr HT, Paulson HL, Yang L, Kotin RM, Davidson BL. RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia. Nature Medicine. 10: 816-20. PMID 15235598 DOI: 10.1038/nm1076  1
2004 Orr HT. RNA gains a new function: a mediator of neurodegeneration. Trends in Neurosciences. 27: 233-4. PMID 15111000 DOI: 10.1016/j.tins.2004.03.003  1
2004 Brodie CR, Khaliq M, Yin JC, Brent Clark H, Orr HT, Boland LM. Overexpression of CREB reduces CRE-mediated transcription: behavioral and cellular analyses in transgenic mice. Molecular and Cellular Neurosciences. 25: 602-11. PMID 15080890 DOI: 10.1016/j.mcn.2003.11.008  1
2004 Opal P, Garcia JJ, McCall AE, Xu B, Weeber EJ, Sweatt JD, Orr HT, Zoghbi HY. Generation and characterization of LANP/pp32 null mice. Molecular and Cellular Biology. 24: 3140-9. PMID 15060138 DOI: 10.1128/MCB.24.8.3140-3149.2004  1
2004 Orr HT. Into the depths of ataxia. The Journal of Clinical Investigation. 113: 505-7. PMID 14966557 DOI: 10.1172/JCI21092  1
2003 Watase K, Venken KJ, Sun Y, Orr HT, Zoghbi HY. Regional differences of somatic CAG repeat instability do not account for selective neuronal vulnerability in a knock-in mouse model of SCA1. Human Molecular Genetics. 12: 2789-95. PMID 12952864 DOI: 10.1093/hmg/ddg300  1
2003 Opal P, Garcia JJ, Propst F, Matilla A, Orr HT, Zoghbi HY. Mapmodulin/leucine-rich acidic nuclear protein binds the light chain of microtubule-associated protein 1B and modulates neuritogenesis. The Journal of Biological Chemistry. 278: 34691-9. PMID 12807913 DOI: 10.1074/jbc.M302785200  1
2003 Chen HK, Fernandez-Funez P, Acevedo SF, Lam YC, Kaytor MD, Fernandez MH, Aitken A, Skoulakis EM, Orr HT, Botas J, Zoghbi HY. Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell. 113: 457-68. PMID 12757707 DOI: 10.1016/S0092-8674(03)00349-0  1
2003 Emamian ES, Kaytor MD, Duvick LA, Zu T, Tousey SK, Zoghbi HY, Clark HB, Orr HT. Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Neuron. 38: 375-87. PMID 12741986 DOI: 10.1016/S0896-6273(03)00258-7  1
2003 Orr HT. Spinocerebellar Ataxia 1 (SCA1) Genetics of Movement Disorders. 35-43. DOI: 10.1016/B978-012566652-7/50005-8  1
2002 Orr HT. Lurcher, nPIST, and autophagy. Neuron. 35: 813-4. PMID 12372273 DOI: 10.1016/S0896-6273(02)00871-1  1
2002 Orr HT. Microarrays and polyglutamine disorders: reports from the Hereditary Disease Array Group. Human Molecular Genetics. 11: 1909-10. PMID 12165553  1
2002 Watase K, Weeber EJ, Xu B, Antalffy B, Yuva-Paylor L, Hashimoto K, Kano M, Atkinson R, Sun Y, Armstrong DL, Sweatt JD, Orr HT, Paylor R, Zoghbi HY. A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration. Neuron. 34: 905-19. PMID 12086639 DOI: 10.1016/S0896-6273(02)00733-X  1
2002 Kaytor MD, Orr HT. The GSK3 beta signaling cascade and neurodegenerative disease. Current Opinion in Neurobiology. 12: 275-8. PMID 12049933 DOI: 10.1016/S0959-4388(02)00320-3  1
2002 Skinner PJ, Vierra-Green CA, Emamian E, Zoghbi HY, Orr HT. Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice. Neuromolecular Medicine. 1: 33-42. PMID 12025814 DOI: 10.1385/NMM:1:1:33  1
2002 Orr HT. A proposed mechanism of ALS fails the test in vivo. Nature Neuroscience. 5: 287-8. PMID 11914714 DOI: 10.1038/nn0402-287  1
2001 Orr HT. Hereditary ataxia. An unfolded protein. Lancet. 358: S35. PMID 11784584  1
2001 Shahbazian MD, Orr HT, Zoghbi HY. Reduction of Purkinje cell pathology in SCA1 transgenic mice by p53 deletion. Neurobiology of Disease. 8: 974-81. PMID 11741393 DOI: 10.1006/nbdi.2001.0444  1
2001 Kaytor MD, Orr HT. RNA targets of the fragile X protein. Cell. 107: 555-7. PMID 11733054 DOI: 10.1016/S0092-8674(01)00590-6  1
2001 Orr HT, Zoghbi HY. SCA1 molecular genetics: a history of a 13 year collaboration against glutamines. Human Molecular Genetics. 10: 2307-11. PMID 11673415  1
2001 Orr HT. Qs in the nucleus. Neuron. 31: 875-6. PMID 11580886 DOI: 10.1016/S0896-6273(01)00435-4  1
2001 Skinner PJ, Vierra-Green CA, Clark HB, Zoghbi HY, Orr HT. Altered trafficking of membrane proteins in purkinje cells of SCA1 transgenic mice. The American Journal of Pathology. 159: 905-13. PMID 11549583 DOI: 10.1016/S0002-9440(10)61766-X  1
2001 Cummings CJ, Sun Y, Opal P, Antalffy B, Mestril R, Orr HT, Dillmann WH, Zoghbi HY. Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Human Molecular Genetics. 10: 1511-8. PMID 11448943  1
2001 Orr HT. Beyond the Qs in the polyglutamine diseases. Genes & Development. 15: 925-32. PMID 11316786 DOI: 10.1101/gad.888401  1
2001 Inoue T, Lin X, Kohlmeier KA, Orr HT, Zoghbi HY, Ross WN. Calcium dynamics and electrophysiological properties of cerebellar Purkinje cells in SCA1 transgenic mice. Journal of Neurophysiology. 85: 1750-60. PMID 11287496  1
2001 Yue S, Serra HG, Zoghbi HY, Orr HT. The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract. Human Molecular Genetics. 10: 25-30. PMID 11136710  1
2001 Davidson JD, Orr HT. Expanding our understanding of polyglutamine disease through transgenic mice Current Genomics. 2: 27-39. DOI: 10.2174/1389202013351183  1
2000 Davidson JD, Riley B, Burright EN, Duvick LA, Zoghbi HY, Orr HT. Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein. Human Molecular Genetics. 9: 2305-12. PMID 11001934  1
2000 Orr HT. The ins and outs of a polyglutamine neurodegenerative disease: spinocerebellar ataxia type 1 (SCA1). Neurobiology of Disease. 7: 129-34. PMID 10860780 DOI: 10.1006/nbdi.2000.0305  1
2000 Zoghbi HY, Orr HT. Glutamine repeats and neurodegeneration. Annual Review of Neuroscience. 23: 217-47. PMID 10845064 DOI: 10.1146/annurev.neuro.23.1.217  1
2000 Orr HT, Zoghbi HY. Reversing neurodegeneration: a promise unfolds. Cell. 101: 1-4. PMID 10778849 DOI: 10.1016/S0092-8674(00)80617-0  1
2000 Clark HB, Orr HT. Spinocerebellar ataxia type 1--modeling the pathogenesis of a polyglutamine neurodegenerative disorder in transgenic mice. Journal of Neuropathology and Experimental Neurology. 59: 265-70. PMID 10759181 DOI: 10.1016/B978-012088592-3/50015-3  1
2000 Lorenzetti D, Watase K, Xu B, Matzuk MM, Orr HT, Zoghbi HY. Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus. Human Molecular Genetics. 9: 779-85. PMID 10749985  1
2000 Lin X, Antalffy B, Kang D, Orr HT, Zoghbi HY. Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1. Nature Neuroscience. 3: 157-63. PMID 10649571 DOI: 10.1038/72101  1
1999 Cummings CJ, Reinstein E, Sun Y, Antalffy B, Jiang Y, Ciechanover A, Orr HT, Beaudet AL, Zoghbi HY. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron. 24: 879-92. PMID 10624951  1
1999 Zoghbi HY, Orr HT. Polyglutamine diseases: protein cleavage and aggregation. Current Opinion in Neurobiology. 9: 566-70. PMID 10508741 DOI: 10.1016/S0959-4388(99)00013-6  1
1999 Kaytor MD, Duvick LA, Skinner PJ, Koob MD, Ranum LP, Orr HT. Nuclear localization of the spinocerebellar ataxia type 7 protein, ataxin-7. Human Molecular Genetics. 8: 1657-64. PMID 10441328 DOI: 10.1093/hmg/8.9.1657  1
1999 Cummings CJ, Orr HT, Zoghbi HY. Progress in pathogenesis studies of spinocerebellar ataxia type 1. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 354: 1079-81. PMID 10434309 DOI: 10.1098/rstb.1999.0462  1
1999 Klement IA, Zoghbi HY, Orr HT. Pathogenesis of polyglutamine-induced disease: A model for SCA1. Molecular Genetics and Metabolism. 66: 172-8. PMID 10066385 DOI: 10.1006/mgme.1999.2801  1
1998 Klement IA, Skinner PJ, Kaytor MD, Yi H, Hersch SM, Clark HB, Zoghbi HY, Orr HT. Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell. 95: 41-53. PMID 9778246 DOI: 10.1016/S0092-8674(00)81781-X  1
1998 Athanasiou MC, Yunis W, Coleman N, Ehlenfeldt R, Clark HB, Orr HT, Feddersen RM. The transcription factor E2F-1 in SV40 T antigen-induced cerebellar Purkinje cell degeneration. Molecular and Cellular Neurosciences. 12: 16-28. PMID 9770337 DOI: 10.1006/mcne.1998.0699  1
1998 Matilla A, Roberson ED, Banfi S, Morales J, Armstrong DL, Burright EN, Orr HT, Sweatt JD, Zoghbi HY, Matzuk MM. Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 18: 5508-16. PMID 9651231  1
1998 Cummings CJ, Mancini MA, Antalffy B, DeFranco DB, Orr HT, Zoghbi HY. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nature Genetics. 19: 148-54. PMID 9620770 DOI: 10.1038/502  1
1997 Schmidt CM, Garrett E, Orr HT. Cytotoxic T lymphocyte recognition of HLA-G in mice. Human Immunology. 55: 127-39. PMID 9361964 DOI: 10.1016/S0198-8859(97)00097-9  1
1997 Matilla A, Koshy BT, Cummings CJ, Isobe T, Orr HT, Zoghbi HY. The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1. Nature. 389: 974-8. PMID 9353121 DOI: 10.1038/40159  1
1997 Skinner PJ, Koshy BT, Cummings CJ, Klement IA, Helin K, Servadio A, Zoghbi HY, Orr HT. Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures. Nature. 389: 971-4. PMID 9353120 DOI: 10.1038/40153  1
1997 Kaytor MD, Burright EN, Duvick LA, Zoghbi HY, Orr HT. Increased trinucleotide repeat instability with advanced maternal age. Human Molecular Genetics. 6: 2135-9. PMID 9328478 DOI: 10.1093/hmg/6.12.2135  1
1997 Clark HB, Burright EN, Yunis WS, Larson S, Wilcox C, Hartman B, Matilla A, Zoghbi HY, Orr HT. Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 17: 7385-95. PMID 9295384  1
1997 Burright EN, Orr HT, Clark HB. Mouse models of human CAG repeat disorders. Brain Pathology (Zurich, Switzerland). 7: 965-77. PMID 9217978  1
1997 Feddersen RM, Yunis WS, O'Donnell MA, Ebner TJ, Shen L, Iadecola C, Orr HT, Clark HB. Susceptibility to cell death induced by mutant SV40 T-antigen correlates with Purkinje neuron functional development. Molecular and Cellular Neurosciences. 9: 42-62. PMID 9204479 DOI: 10.1006/mcne.1997.0601  1
1997 Burright EN, Davidson JD, Duvick LA, Koshy B, Zoghbi HY, Orr HT. Identification of a self-association region within the SCA1 gene product, ataxin-1. Human Molecular Genetics. 6: 513-8. PMID 9097953 DOI: 10.1093/hmg/6.4.513  1
1997 Zoghbi HY, Orr HT. The molecular genetics of spinocerebellar degenerations Faseb Journal. 11: A1004.  1
1996 Orr HT, Zoghbi HY. Toward understanding polyglutamine-induced neurological disease in spinocerebellar ataxia type 1. Cold Spring Harbor Symposia On Quantitative Biology. 61: 649-57. PMID 9246491  1
1996 Pavlou O, Ehlenfeldt R, Horn S, Orr HT. Isolation, characterization and in vivo analysis of the murine calbindin-D28K upstream regulatory region. Brain Research. Molecular Brain Research. 36: 268-79. PMID 8965647 DOI: 10.1016/0169-328X(95)00259-U  1
1996 Koshy B, Matilla T, Burright EN, Merry DE, Fischbeck KH, Orr HT, Zoghbi HY. Spinocerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase. Human Molecular Genetics. 5: 1311-8. PMID 8872471 DOI: 10.1093/hmg/5.9.1311  1
1996 Banfi S, Servadio A, Chung M, Capozzoli F, Duvick LA, Elde R, Zoghbi HY, Orr HT. Cloning and developmental expression analysis of the murine homolog of the spinocerebellar ataxia type 1 gene (Sca1). Human Molecular Genetics. 5: 33-40. PMID 8789437 DOI: 10.1093/hmg/5.1.33  1
1995 Schmidt CM, Orr HT. HLA-G transgenic mice: a model for studying expression and function at the maternal/fetal interface. Immunological Reviews. 147: 53-65. PMID 8847080  1
1995 Dubovsky J, Zabramski JM, Kurth J, Spetzler RF, Rich SS, Orr HT, Weber JL. A gene responsible for cavernous malformations of the brain maps to chromosome 7q. Human Molecular Genetics. 4: 453-8. PMID 7795602 DOI: 10.1093/hmg/4.3.453  1
1995 Chong SS, McCall AE, Cota J, Subramony SH, Orr HT, Hughes MR, Zoghbi HY. Homozygotes for CDKN2 (p16) germline mutation in Dutch familial melanoma kindreds Nature Genetics. 10: 351-353. PMID 7670475 DOI: 10.1038/ng0795-351  1
1995 Chong SS, McCall AE, Cota J, Subramony SH, Orr HT, Hughes MR, Zoghbi HY. Gametic and somatic tissue-specific heterogeneity of the expanded SCA1 CAG repeat in spinocerebellar ataxia type 1. Nature Genetics. 10: 344-50. PMID 7670474 DOI: 10.1038/ng0795-344  1
1995 Ranum LP, Lundgren JK, Schut LJ, Ahrens MJ, Perlman S, Aita J, Bird TD, Gomez C, Orr HT. Spinocerebellar ataxia type 1 and Machado-Joseph disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia. American Journal of Human Genetics. 57: 603-8. PMID 7668288  1
1995 Servadio A, Koshy B, Armstrong D, Antalffy B, Orr HT, Zoghbi HY. Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals. Nature Genetics. 10: 94-8. PMID 7647801 DOI: 10.1038/ng0595-94  1
1995 Orr HT, Clark HB. Genetic approaches to pathogenesis of neurodegenerative diseases. Laboratory Investigation; a Journal of Technical Methods and Pathology. 73: 161-71. PMID 7637317  1
1995 Zoghbi HY, Orr HT. Spinocerebellar ataxia type 1. Seminars in Cell Biology. 6: 29-35. PMID 7620119 DOI: 10.1016/1043-4682(95)90012-8  1
1995 Schmidt CM, Chen HL, Chiu I, Ehlenfeldt RG, Hunt JS, Orr HT. Temporal and spatial expression of HLA-G messenger RNA in extraembryonic tissues of transgenic mice. Journal of Immunology (Baltimore, Md. : 1950). 155: 619-29. PMID 7608540  1
1995 Burright EN, Clark HB, Servadio A, Matilla T, Feddersen RM, Yunis WS, Duvick LA, Zoghbi HY, Orr HT. SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell. 82: 937-48. PMID 7553854 DOI: 10.1016/0092-8674(95)90273-2  1
1995 Feddersen RM, Clark HB, Yunis WS, Orr HT. In vivo viability of postmitotic Purkinje neurons requires pRb family member function. Molecular and Cellular Neurosciences. 6: 153-67. PMID 7551567 DOI: 10.1006/mcne.1995.1014  1
1994 Volz A, Boyle JM, Cann HM, Cottingham RW, Orr HT, Ziegler A. Report of the Second International Workshop on Human Chromosome 6. Genomics. 21: 464-72. PMID 8088851 DOI: 10.1006/geno.1994.1302  1
1994 Orr HT. Unstable trinucleotide repeats and the diagnosis of neurodegenerative disease. Human Pathology. 25: 598-601. PMID 8013951 DOI: 10.1016/0046-8177(94)90226-7  1
1994 Banfi S, Servadio A, Chung MY, Kwiatkowski TJ, McCall AE, Duvick LA, Shen Y, Roth EJ, Orr HT, Zoghbi HY. Identification and characterization of the gene causing type 1 spinocerebellar ataxia. Nature Genetics. 7: 513-20. PMID 7951322 DOI: 10.1038/ng0894-513  1
1994 Ranum LP, Schut LJ, Lundgren JK, Orr HT, Livingston DM. Spinocerebellar ataxia type 5 in a family descended from the grandparents of President Lincoln maps to chromosome 11. Nature Genetics. 8: 280-4. PMID 7874171 DOI: 10.1038/ng1194-280  1
1993 Zoghbi HY, Frontali M, Orr HT, Sandkuijl L, Cann H, Sasaki H, Chamberlain S, Terrenato L, Rich SS. Linkage studies in dominantly inherited ataxias. Advances in Neurology. 61: 133-7. PMID 8421963  1
1993 Yelavarthi KK, Schmidt CM, Ehlenfeldt RG, Orr HT, Hunt JS. Cellular distribution of HLA-G mRNA in transgenic mouse placentas. Journal of Immunology (Baltimore, Md. : 1950). 151: 3638-45. PMID 8376798  1
1993 Schmidt CM, Ehlenfeldt RG, Athanasiou MC, Duvick LA, Heinrichs H, David CS, Orr HT. Extraembryonic expression of the human MHC class I gene HLA-G in transgenic mice. Evidence for a positive regulatory region located 1 kilobase 5' to the start site of transcription. Journal of Immunology (Baltimore, Md. : 1950). 151: 2633-45. PMID 8360483  1
1993 Orr HT, Chung MY, Banfi S, Kwiatkowski TJ, Servadio A, Beaudet AL, McCall AE, Duvick LA, Ranum LP, Zoghbi HY. Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nature Genetics. 4: 221-6. PMID 8358429 DOI: 10.1038/ng0793-221  1
1993 Banfi S, Chung MY, Kwiatkowski TJ, Ranum LP, McCall AE, Chinault AC, Orr HT, Zoghbi HY. Mapping and cloning of the critical region for the spinocerebellar ataxia type 1 gene (SCA1) in a yeast artificial chromosome contig spanning 1.2 Mb. Genomics. 18: 627-35. PMID 8307572 DOI: 10.1016/S0888-7543(05)80365-9  1
1993 Chung MY, Ranum LP, Duvick LA, Servadio A, Zoghbi HY, Orr HT. Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I. Nature Genetics. 5: 254-8. PMID 8275090 DOI: 10.1038/ng1193-254  1
1993 Schmidt CM, Orr HT. Maternal/fetal interactions: the role of the MHC class I molecule HLA-G. Critical Reviews in Immunology. 13: 207-24. PMID 8110376  1
1992 Ranum LP, Rich SS, Nance MA, Duvick LA, Aita JF, Orr HT, Anton-Johnson S, Schut LJ. Autosomal dominant spinocerebellar ataxia: locus heterogeneity in a Nebraska kindred. Neurology. 42: 344-7. PMID 1736163  1
1992 Messer G, Zemmour J, Orr HT, Parham P, Weiss EH, Girdlestone J. HLA-J, a second inactivated class I HLA gene related to HLA-G and HLA-A. Implications for the evolution of the HLA-A-related genes. Journal of Immunology (Baltimore, Md. : 1950). 148: 4043-53. PMID 1602142  1
1992 Hunt JS, Orr HT. HLA and maternal-fetal recognition. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 6: 2344-8. PMID 1544544  1
1992 Geraghty DE, Koller BH, Hansen JA, Orr HT. The HLA class I gene family includes at least six genes and twelve pseudogenes and gene fragments. Journal of Immunology (Baltimore, Md. : 1950). 149: 1934-46. PMID 1517563  1
1992 Feddersen RM, Ehlenfeldt R, Yunis WS, Clark HB, Orr HT. Disrupted cerebellar cortical development and progressive degeneration of Purkinje cells in SV40 T antigen transgenic mice. Neuron. 9: 955-66. PMID 1419002 DOI: 10.1016/0896-6273(92)90247-B  1
1991 Vandaele S, Nordquist DT, Feddersen RM, Tretjakoff I, Peterson AC, Orr HT. Purkinje cell protein-2 regulatory regions and transgene expression in cerebellar compartments. Genes & Development. 5: 1136-48. PMID 2065970  1
1991 Ranum LP, Chung MY, Duvick LA, Zoghbi HY, Orr HT. Dinucleotide repeat polymorphism at the D6S109 locus. Nucleic Acids Research. 19: 1171. PMID 2020562 DOI: 10.1093/nar/19.5.1171-a  1
1991 Killeen AA, Sane KS, Orr HT. Molecular and endocrine characterization of a mutation involving a recombination between the steroid 21-hydroxylase functional gene and pseudogene. The Journal of Steroid Biochemistry and Molecular Biology. 38: 677-86. PMID 1905948 DOI: 10.1016/0960-0760(91)90078-J  1
1991 Schmidt CM, Orr HT. A physical linkage map of HLA-A, -G, -7.5p, and -F. Human Immunology. 31: 180-5. PMID 1890019 DOI: 10.1016/0198-8859(91)90024-4  1
1991 Nance MA, Leroy BS, Orr HT, Parker T, Rich SS, Heston LL. Protocol for genetic testing in Huntington disease: three years of experience in Minnesota. American Journal of Medical Genetics. 40: 518-22. PMID 1836107 DOI: 10.1002/ajmg.1320400431  1
1991 Heston LL, Orr HT, Rich SS, White JA. Linkage of an Alzheimer disease susceptibility locus to markers on human chromosome 21. American Journal of Medical Genetics. 40: 449-53. PMID 1684090 DOI: 10.1002/ajmg.1320400415  1
1991 Ranum LP, Duvick LA, Rich SS, Schut LJ, Litt M, Orr HT. Localization of the autosomal dominant HLA-linked spinocerebellar ataxia (SCA1) locus, in two kindreds, within an 8-cM subregion of chromosome 6p. American Journal of Human Genetics. 49: 31-41. PMID 1676561  1
1990 Zemmour J, Koller BH, Ennis PD, Geraghty DE, Lawlor DA, Orr HT, Parham P. HLA-AR, an inactivated antigen-presenting locus related to HLA-A. Implications for the evolution of the MHC. Journal of Immunology (Baltimore, Md. : 1950). 144: 3619-29. PMID 2329283  1
1990 Chen SL, Orr HT. Sequence of a murine cDNA, pcp-4, that encodes the homolog of the rat brain-specific antigen PEP-19. Nucleic Acids Research. 18: 1304. PMID 2320430 DOI: 10.1093/nar/18.5.1304  1
1990 Wei XH, Orr HT. Differential expression of HLA-E, HLA-F, and HLA-G transcripts in human tissue. Human Immunology. 29: 131-42. PMID 2249951 DOI: 10.1016/0198-8859(90)90076-2  1
1990 Heinrichs H, Orr HT. HLA non-A,B,C class I genes: their structure and expression. Immunologic Research. 9: 265-74. PMID 2089074 DOI: 10.1007/BF02935526  1
1990 Duvick L, Rich SS, Orr HT. A polymorphic DNA probe, p1-10-2, from chromosome 6. Nucleic Acids Research. 18: 3105. PMID 1971928 DOI: 10.1093/nar/18.10.3105  1
1990 Geraghty DE, Wei XH, Orr HT, Koller BH. Human leukocyte antigen F (HLA-F). An expressed HLA gene composed of a class I coding sequence linked to a novel transcribed repetitive element. The Journal of Experimental Medicine. 171: 1-18. PMID 1688605  1
1989 Zachow KR, Orr HT. Regulation of HLA class I transcription in T cells. Journal of Immunology (Baltimore, Md. : 1950). 143: 3385-9. PMID 2809207  1
1989 Koller BH, Geraghty DE, DeMars R, Duvick L, Rich SS, Orr HT. Chromosomal organization of the human major histocompatibility complex class I gene family. The Journal of Experimental Medicine. 169: 469-80. PMID 2562983  1
1989 Orr HT. Molecular genetics of the human leukocyte antigen complex. Immunology Series. 43: 319-38. PMID 2490501  1
1988 Choo SY, St John T, Orr HT, Hansen JA. Molecular analysis of the variant alloantigen HLA-B27d (HLA-B*2703) identifies a unique single amino acid substitution. Human Immunology. 21: 209-19. PMID 3286582 DOI: 10.1016/0198-8859(88)90072-9  1
1988 Koller BH, Geraghty DE, Shimizu Y, DeMars R, Orr HT. HLA-E. A novel HLA class I gene expressed in resting T lymphocytes. Journal of Immunology (Baltimore, Md. : 1950). 141: 897-904. PMID 3260916  1
1988 Shimizu Y, Geraghty DE, Koller BH, Orr HT, DeMars R. Transfer and expression of three cloned human non-HLA-A,B,C class I major histocompatibility complex genes in mutant lymphoblastoid cells. Proceedings of the National Academy of Sciences of the United States of America. 85: 227-31. PMID 3257565  1
1988 Nordquist DT, Kozak CA, Orr HT. cDNA cloning and characterization of three genes uniquely expressed in cerebellum by Purkinje neurons. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 8: 4780-9. PMID 3199205  1
1988 Orr HT, Parker T, Wielinski CL, Clawson CC, Warwick WJ. Cystic fibrosis: chromosome 7 DNA genotyping. An aide in resolving ambiguous diagnoses in siblings of known patients. Clinical Pediatrics. 27: 591-5. PMID 3191652 DOI: 10.1177/000992288802701205  1
1988 Killeen AA, Seelig S, Ulstrom RA, Orr HT. Diagnosis of classical steroid 21-hydroxylase deficiency using an HLA-B locus-specific DNA-probe. American Journal of Medical Genetics. 29: 703-12. PMID 2897792 DOI: 10.1002/ajmg.1320290340  1
1987 Geraghty DE, Koller BH, Orr HT. A human major histocompatibility complex class I gene that encodes a protein with a shortened cytoplasmic segment. Proceedings of the National Academy of Sciences of the United States of America. 84: 9145-9. PMID 3480534  1
1987 Rich SS, Wilkie P, Schut L, Vance G, Orr HT. Spinocerebellar ataxia: localization of an autosomal dominant locus between two markers on human chromosome 6. American Journal of Human Genetics. 41: 524-31. PMID 3477955  1
1987 Koller BH, Geraghty D, Orr HT, Shimizu Y, DeMars R. Organization of the human class I major histocompatibility complex genes. Immunologic Research. 6: 1-10. PMID 3040873 DOI: 10.1007/BF02918100  1
1987 Carroll MC, Katzman P, Alicot EM, Koller BH, Geraghty DE, Orr HT, Strominger JL, Spies T. Linkage map of the human major histocompatibility complex including the tumor necrosis factor genes. Proceedings of the National Academy of Sciences of the United States of America. 84: 8535-9. PMID 2825194  1
1987 Jackson JB, Orr HT, McCullough JJ, Jordan MC. Failure to detect human cytomegalovirus DNA in IgM-seropositive blood donors by spot hybridization. The Journal of Infectious Diseases. 156: 1013-6. PMID 2824620 DOI: 10.1093/infdis/156.6.1013  1
1986 Blazar BR, Lasky LC, Perentesis JP, Watson KV, Steinberg SE, Filipovich AH, Orr HT, Ramsay NK. Successful donor cell engraftment in a recipient of bone marrow from a cadaveric donor. Blood. 67: 1655-60. PMID 3518833  1
1986 Steere K, Sidwell B, Leach R, Ward FE, Taurog JD, Orr HT. Use of DNA probes from the 5' flanking region of the HLA-B gene to examine polymorphism at the HLA-B locus. Human Immunology. 16: 137-47. PMID 3013813 DOI: 10.1016/0198-8859(86)90042-X  1
1986 Blazar BR, Whitley CB, Desnick RJ, Pierpont ME, Filipovich AH, Ramsay NK, Kersey JH, Orr HT, Yasmineh WG, Tsai MY. Comparison of enzymatic activity with evidence of engraftment in patients with inborn errors of metabolism receiving allogeneic marrow transplantation. Birth Defects Original Article Series. 22: 135-52. PMID 2870747  1
1985 DeMars R, Rudersdorf R, Chang C, Petersen J, Strandtmann J, Korn N, Sidwell B, Orr HT. Mutations that impair a posttranscriptional step in expression of HLA-A and -B antigens. Proceedings of the National Academy of Sciences of the United States of America. 82: 8183-7. PMID 3906658 DOI: 10.1073/pnas.82.23.8183  1
1985 Blazar BR, Orr HT, Arthur DC, Kersey JH, Filipovich AH. Restriction fragment length polymorphisms as markers of engraftment in allogeneic marrow transplantation. Blood. 66: 1436-44. PMID 2998513  1
1985 Koller BH, Orr HT. Cloning and complete sequence of an HLA-A2 gene: analysis of two HLA-A alleles at the nucleotide level. Journal of Immunology (Baltimore, Md. : 1950). 134: 2727-33. PMID 2982951  1
1985 Koller BH, Ward FE, DeMars R, Orr HT. Comparison of multiple HLA-A alleles at the DNA level by using Southern blotting and HLA-A-specific probes. Journal of Immunology (Baltimore, Md. : 1950). 135: 4229-34. PMID 2415606  1
1984 Koller BH, Sidwell B, DeMars R, Orr HT. Isolation of HLA locus-specific DNA probes from the 3'-untranslated region. Proceedings of the National Academy of Sciences of the United States of America. 81: 5175-8. PMID 6591185  1
1984 Blazar BR, Filipovich AH, Orr HT. Use of DNA polymorphic markers to determine the chimeric status of matched and mismatched patients (pts) following bone marrow transplantation (BMT) Federation Proceedings. 43: no. 3306.  1
1983 Orr HT, DeMars R. Mapping of class I DNA sequences within the human major histocompatibility complex. Immunogenetics. 18: 489-502. PMID 6315574 DOI: 10.1007/BF00364390  1
1983 Orr HT, DeMars R. Class I-like HLA genes map telomeric to the HLA-A2 locus in human cells. Nature. 302: 534-6. PMID 6300687 DOI: 10.1038/302534a0  1
1983 Orr HT. Use of Southern blotting to analyze the size and restriction fragment polymorphism of HLA class I DNA in the human population Transplantation Proceedings. 15: 1900-1906.  1
1982 Orr HT, Bach FH, Ploegh HL, Strominger JL, Kavathas P, DeMars R. Use of HLA loss mutants to analyse the structure of the human major histocompatibility complex. Nature. 296: 454-6. PMID 7063041 DOI: 10.1038/296454a0  1
1982 López de Castro JA, Strominger JL, Strong DM, Orr HT. Structure of crossreactive human histocompatibility antigens HLA-A28 and HLA-A2: possible implications for the generation of HLA polymorphism. Proceedings of the National Academy of Sciences of the United States of America. 79: 3813-7. PMID 6179086  1
1981 Ploegh HL, Orr HT, Strominger JL. Major histocompatibility antigens: the human (HLA-A, -B, -C) and murine (H-2K, H-2D) class I molecules. Cell. 24: 287-99. PMID 7016338 DOI: 10.1016/0092-8674(81)90318-4  1
1981 Ploegh HL, Orr HT, Stominger JL. Biosynthesis and cell surface localization of nonglycosylated human histocompatibility antigens. Journal of Immunology (Baltimore, Md. : 1950). 126: 270-5. PMID 7005335  1
1980 Strominger JL, Engelhard VH, Guild BC, Kostyk TG, Lancet D, Lopez de Castro JA, Orr HT, Parham P, Ploegh HL, Pober JS. Complete primary structure of human histocompatibility antigen HLA-B7: evolutionary and functional implications. Current Topics in Developmental Biology. 14: 97-113. PMID 7460613 DOI: 10.1016/S0070-2153(08)60190-8  1
1980 Strominger JL, Orr HT, Parham P, Ploegh HL, Mann DL, Bilofsky H, Saroff HA, Wu TT, Kabat EA. An evaluation of the significance of amino acid sequence homologies in human histocompatibility antigens (HLA-A and HLA-B) with immunoglobulins and other proteins, using relatively short sequences. Scandinavian Journal of Immunology. 11: 573-92. PMID 7444380  1
1980 Krangel MS, Orr HT, Strominger JL. Structure, function, and biosynthesis of the major human histocompatibility antigens (HLA-A and HLA-B). Scandinavian Journal of Immunology. 11: 561-71. PMID 7444379  1
1980 Ploegh HL, Orr HT, Strominger JL. Molecular cloning of a human histocompatibility antigen cDNA fragment. Proceedings of the National Academy of Sciences of the United States of America. 77: 6081-5. PMID 6934534  1
1979 Orr HT, López de Castro JA, Lancet D, Strominger JL. Complete amino acid sequence of a papain-solubilized human histocompatibility antigen, HLA-B7. 2. Sequence determination and search for homologies. Biochemistry. 18: 5711-20. PMID 518865  1
1979 López de Castro JA, Orr HT, Robb RJ, Kostyk TG, Mann DL, Strominger JL. Complete amino acid sequence of a papain-solubilized human histocompatibility antigen HLA-B7. 1. Isolation and amino acid composition of fragments and of tryptic and chymotryptic peptides. Biochemistry. 18: 5704-11. PMID 518864  1
1979 Orr HT, Lancet D, Robb RJ, Lopez de Castro JA, Strominger JL. The heavy chain of human histocompatibility antigen HLA-B7 contains an immunoglobulin-like region. Nature. 282: 266-70. PMID 388231 DOI: 10.1038/282266a0  1
1979 Krangel MS, Orr HT, Strominger JL. Assembly and maturation of HLA-A and HLA-B antigens in vivo. Cell. 18: 979-91. PMID 93026 DOI: 10.1016/0092-8674(79)90210-1  1
1979 Orr HT, Lopez de Castro JA, Parham P, Ploegh HL, Strominger JL. Comparison of amino acid sequences of two human histocompatibility antigens, HLA-A2 and HLA-B7: location of putative alloantigenic sites. Proceedings of the National Academy of Sciences of the United States of America. 76: 4395-9. PMID 92029  1
1977 Fuks A, Kaufman JF, Orr HT, Parham P, Robb RR, Terhorst C, Strominger JL. Structural aspects of the products of the human major histocompatibility complex. Transplantation Proceedings. 9: 1685-9. PMID 304615  1
1977 Ferrendelli JA, Rubin EH, Orr HT, Kinscherf DA, Lowry OH. Measurement of cyclic nucleotides in histologically defined samples of brain and retina. Analytical Biochemistry. 78: 252-9. PMID 192102 DOI: 10.1016/0003-2697(77)90030-6  1
1977 Parham P, Alpert BN, Orr HT, Strominger JL. Carbohydrate moiety of HLA antigens. Antigenic properties and amino acid sequences around the site of glycosylation. The Journal of Biological Chemistry. 252: 7555-67. PMID 72068  1
1976 Orr HT, Cohen AI, Carter JA. The levels of free taurine, glutamate, glycine and gamma-amino butyric acid during the postnatal development of the normal and dystrophic retina of the mouse. Experimental Eye Research. 23: 377-84. PMID 976379 DOI: 10.1016/0014-4835(76)90165-2  1
1976 Orr HT, Cohen AI, Lowry OH. The distribution of taurine in the vertebrate retina. Journal of Neurochemistry. 26: 609-11. PMID 816997  1
1976 Orr HT, Lowry OH, Cohen AI, Ferrendelli JA. Distribution of 3':5'-cyclic AMP and 3':5'-cyclic GMP in rabbit retina in vivo: selective effects of dark and light adaptation and ischemia. Proceedings of the National Academy of Sciences of the United States of America. 73: 4442-5. PMID 188039  1
1972 Riley MV, Hodson SA, Orr HT. Synthetic activity of corneal endothelium. Israel Journal of Medical Sciences. 8: 1519-22. PMID 4265111  1
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