| Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1 HT Orr, M Chung, S Banfi, TJ Kwiatkowski Jr, A Servadio, AL Beaudet, ... Nature genetics 4 (3), 221-226, 1993 | 2034 | 1993 |
| Trinucleotide repeat disorders HT Orr, HY Zoghbi Annu. Rev. Neurosci. 30 (1), 575-621, 2007 | 1661 | 2007 |
| Glutamine repeats and neurodegeneration HY Zoghbi, HT Orr Annual review of neuroscience 23 (1), 217-247, 2000 | 1639 | 2000 |
| Genetic linkage evidence for a familial Alzheimer's disease locus on chromosome 14 GD Schellenberg, TD Bird, EM Wijsman, HT Orr, L Anderson, E Nemens, ... Science 258 (5082), 668-671, 1992 | 1374 | 1992 |
| Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice IA Klement, PJ Skinner, MD Kaytor, H Yi, SM Hersch, HB Clark, ... Cell 95 (1), 41-53, 1998 | 1167 | 1998 |
| Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1 CJ Cummings, MA Mancini, B Antalffy, DB DeFranco, HT Orr, HY Zoghbi Nature genetics 19 (2), 148-154, 1998 | 1062 | 1998 |
| RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia H Xia, Q Mao, SL Eliason, SQ Harper, IH Martins, HT Orr, HL Paulson, ... Nature medicine 10 (8), 816-820, 2004 | 827 | 2004 |
| Identification of genes that modify ataxin-1-induced neurodegeneration P Fernandez-Funez, ML Nino-Rosales, B De Gouyon, WC She, ... Nature 408 (6808), 101-106, 2000 | 784 | 2000 |
| SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat EN Burright, HB Clark, A Servadio, T Matilla, RM Feddersen, WS Yunis, ... Cell 82 (6), 937-948, 1995 | 728 | 1995 |
| A human major histocompatibility complex class I gene that encodes a protein with a shortened cytoplasmic segment. DE Geraghty, BH Koller, HT Orr Proceedings of the national academy of sciences 84 (24), 9145-9149, 1987 | 704 | 1987 |
| Major histocompatibility antigens: the human (HLA-A,-B,-C) and murine (H-2K, H-2D) class I molecules HL Ploegh, HT Orr, JL Strominger Cell 24 (2), 287-299, 1981 | 646 | 1981 |
| Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures PJ Skinner, BT Koshy, CJ Cummings, IA Klement, K Helin, A Servadio, ... Nature 389 (6654), 971-974, 1997 | 634 | 1997 |
| Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice CJ Cummings, Y Sun, P Opal, B Antalffy, R Mestril, HT Orr, WH Dillmann, ... Human molecular genetics 10 (14), 1511-1518, 2001 | 631 | 2001 |
| Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice CJ Cummings, E Reinstein, Y Sun, B Antalffy, Y Jiang, A Ciechanover, ... Neuron 24 (4), 879-892, 1999 | 605 | 1999 |
| Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I M Chung, LPW Ranum, LA Duvick, A Servadio, HY Zoghbi, HT Orr Nature genetics 5 (3), 254-258, 1993 | 592 | 1993 |
| Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1 HK Chen, P Fernandez-Funez, SF Acevedo, YC Lam, MD Kaytor, ... Cell 113 (4), 457-468, 2003 | 514 | 2003 |
| Genetic linkage studies suggest that Alzheimer's disease is not a single homogeneous disorder PH St George-Hyslop, JL Haines, LA Farrer, R Polinsky, CV Broeckhoven, ... Nature 347 (6289), 194-197, 1990 | 512 | 1990 |
| Linkage map of the human major histocompatibility complex including the tumor necrosis factor genes. MC Carroll, P Katzman, EM Alicot, BH Koller, DE Geraghty, HT Orr, ... Proceedings of the National Academy of Sciences 84 (23), 8535-8539, 1987 | 472 | 1987 |
| Identification and characterization of the gene causing type 1 spinocerebellar ataxia S Banfi, A Servadio, M Chung, TJ Kwiatkowski Jr, AE McCall, LA Duvick, ... Nature genetics 7 (4), 513-520, 1994 | 442 | 1994 |
| Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1 X Lin, B Antalffy, D Kang, HT Orr, HY Zoghbi Nature neuroscience 3 (2), 157-163, 2000 | 437 | 2000 |