| Year |
Citation |
Score |
| 2022 |
Coffin SL, Durham MA, Nitschke L, Xhako E, Brown AM, Revelli JP, Villavicencio Gonzalez E, Lin T, Handler HP, Dai Y, Trostle AJ, Wan YW, Liu Z, Sillitoe RV, Orr HT, et al. Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1. Neuron. PMID 36577402 DOI: 10.1016/j.neuron.2022.11.016 |
0.328 |
|
| 2022 |
Lee WS, Al-Ramahi I, Jeong HH, Jang Y, Lin T, Adamski CJ, Lavery LA, Rath S, Richman R, Bondar VV, Alcala E, Revelli JP, Orr HT, Liu Z, Botas J, et al. Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1. The Journal of Clinical Investigation. 132. PMID 35499073 DOI: 10.1172/JCI156616 |
0.301 |
|
| 2021 |
Nitschke L, Coffin SL, Xhako E, El-Najjar DB, Orengo JP, Alcala E, Dai Y, Wan YW, Liu Z, Orr HT, Zoghbi HY. Modulation of ATXN1 S776 phosphorylation reveals the importance of allele-specific targeting in SCA1. Jci Insight. 6. PMID 33554954 DOI: 10.1172/jci.insight.144955 |
0.305 |
|
| 2020 |
O'Callaghan B, Hofstra B, Handler HP, Kordasiewicz HB, Cole T, Duvick L, Friedrich J, Rainwater O, Yang P, Benneyworth M, Nichols-Meade T, Heal W, Ter Haar R, Henzler C, Orr HT. Antisense Oligonucleotide Therapeutic Approach for Suppression of Ataxin-1 Expression: A Safety Assessment. Molecular Therapy. Nucleic Acids. 21: 1006-1016. PMID 32818920 DOI: 10.1016/J.Omtn.2020.07.030 |
0.373 |
|
| 2020 |
Mouro Pinto R, Arning L, Giordano JV, Razghandi P, Andrew MA, Gillis T, Correia K, Mysore JS, Grote Urtubey DM, Parwez CR, Hein von SM, Clark HB, Nguyen HP, Förster E, Beller A, ... ... Orr H, et al. Patterns of CAG repeat instability in the central nervous system and periphery in Huntington's disease and in spinocerebellar ataxia type 1. Human Molecular Genetics. PMID 32761094 DOI: 10.1093/Hmg/Ddaa139 |
0.374 |
|
| 2020 |
Zhang S, Williamson NA, Duvick L, Lee A, Orr HT, Korlin-Downs A, Yang P, Mok YF, Jans DA, Bogoyevitch MA. The ataxin-1 interactome reveals direct connection with multiple disrupted nuclear transport pathways. Nature Communications. 11: 3343. PMID 32620905 DOI: 10.1038/S41467-020-17145-0 |
0.343 |
|
| 2019 |
Chuang CS, Chang JC, Soong BW, Chuang SF, Lin TT, Cheng WL, Orr HT, Liu CS. Treadmill training increases the motor activity and neuron survival of the cerebellum in a mouse model of spinocerebellar ataxia type 1. The Kaohsiung Journal of Medical Sciences. PMID 31271500 DOI: 10.1002/Kjm2.12106 |
0.334 |
|
| 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 |
0.387 |
|
| 2018 |
De Maio A, Yalamanchili HK, Adamski CJ, Gennarino VA, Liu Z, Qin J, Jung SY, Richman R, Orr H, Zoghbi HY. RBM17 Interacts with U2SURP and CHERP to Regulate Expression and Splicing of RNA-Processing Proteins. Cell Reports. 25: 726-736.e7. PMID 30332651 DOI: 10.1016/J.Celrep.2018.09.041 |
0.339 |
|
| 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 |
0.382 |
|
| 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 |
0.774 |
|
| 2018 |
Liu CJ, Rainwater O, Brent Clark H, Orr HT, Akkin T. Polarization-sensitive optical coherence tomography reveals gray matter and white matter atrophy in SCA1 mouse models. Neurobiology of Disease. PMID 29753755 DOI: 10.1016/J.Nbd.2018.05.003 |
0.32 |
|
| 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 |
0.398 |
|
| 2018 |
Pérez Ortiz JM, Orr HT. Spinocerebellar Ataxia Type 1: Molecular Mechanisms of Neurodegeneration and Preclinical Studies. Advances in Experimental Medicine and Biology. 1049: 135-145. PMID 29427101 DOI: 10.1007/978-3-319-71779-1_6 |
0.463 |
|
| 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 |
0.371 |
|
| 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 |
0.336 |
|
| 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 |
0.375 |
|
| 2016 |
Rubinsztein DC, Orr HT. Diminishing return for mechanistic therapeutics with neurodegenerative disease duration?: There may be a point in the course of a neurodegenerative condition where therapeutics targeting disease-causing mechanisms are futile. Bioessays : News and Reviews in Molecular, Cellular and Developmental Biology. PMID 27479863 DOI: 10.1002/Bies.201600048 |
0.348 |
|
| 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 |
0.422 |
|
| 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 |
0.353 |
|
| 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 |
0.379 |
|
| 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 |
0.43 |
|
| 2015 |
Cvetanovic M, Ingram M, Orr H, Opal P. Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1. Neuroscience. 289: 289-99. PMID 25595967 DOI: 10.1016/J.Neuroscience.2015.01.003 |
0.349 |
|
| 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 |
0.313 |
|
| 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 |
0.763 |
|
| 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 |
0.452 |
|
| 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 |
0.763 |
|
| 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 |
0.347 |
|
| 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 |
0.339 |
|
| 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 |
0.352 |
|
| 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 |
0.417 |
|
| 2012 |
Orr HT. Cell biology of spinocerebellar ataxia. The Journal of Cell Biology. 197: 167-77. PMID 22508507 DOI: 10.1083/Jcb.201105092 |
0.414 |
|
| 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 |
0.379 |
|
| 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 |
0.413 |
|
| 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 |
0.323 |
|
| 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 |
0.31 |
|
| 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 |
0.3 |
|
| 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 |
0.358 |
|
| 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 |
0.346 |
|
| 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.A111.238527 |
0.649 |
|
| 2011 |
Orr HT. Are polyglutamine diseases expanding? Neuron. 70: 377-8. PMID 21555065 DOI: 10.1016/J.Neuron.2011.04.015 |
0.308 |
|
| 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 |
0.738 |
|
| 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 |
0.4 |
|
| 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 |
0.428 |
|
| 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 |
0.4 |
|
| 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 |
0.379 |
|
| 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 |
0.738 |
|
| 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 |
0.369 |
|
| 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 |
0.633 |
|
| 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 |
0.435 |
|
| 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 |
0.359 |
|
| 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 |
0.404 |
|
| 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 |
0.375 |
|
| 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 |
0.775 |
|
| 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 |
0.373 |
|
| 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 |
0.351 |
|
| 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 |
0.43 |
|
| 2007 |
Orr HT. 13 – Spinocerebellar Ataxia Type 1 Neurobiology of Disease. 149-155. DOI: 10.1016/B978-012088592-3/50015-3 |
0.464 |
|
| 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 |
0.433 |
|
| 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 |
0.773 |
|
| 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 |
0.395 |
|
| 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 |
0.766 |
|
| 2006 |
Hardy J, Orr H. The genetics of neurodegenerative diseases Journal of Neurochemistry. 97: 1690-1699. PMID 16805777 DOI: 10.1111/J.1471-4159.2006.03979.X |
0.345 |
|
| 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 |
0.421 |
|
| 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 |
0.344 |
|
| 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 |
0.751 |
|
| 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 |
0.414 |
|
| 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 |
0.375 |
|
| 2004 |
Orr HT. Neurodegenerative disease: neuron protection agency. Nature. 431: 747-8. PMID 15483586 DOI: 10.1038/431747A |
0.31 |
|
| 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 |
0.404 |
|
| 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 |
0.409 |
|
| 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 |
0.746 |
|
| 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 |
0.4 |
|
| 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 |
0.75 |
|
| 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 |
0.398 |
|
| 2004 |
Orr HT. Into the depths of ataxia. The Journal of Clinical Investigation. 113: 505-7. PMID 14966557 DOI: 10.1172/Jci21092 |
0.307 |
|
| 2004 |
Xia H, Mao Q, Eliason SL, Kiewiet N, Critchfield J, Martins IH, Harper SQ, He X, Kotin RM, Zoghbi HY, Orr HT, Paulson HL, Davidson BL. 717. RNAi Therapy for Dominant Neurodegenerative Diseases Molecular Therapy. 9. DOI: 10.1016/J.Ymthe.2004.06.623 |
0.393 |
|
| 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 |
0.363 |
|
| 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 |
0.401 |
|
| 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 |
0.442 |
|
| 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 |
0.415 |
|
| 2002 |
Orr HT. Lurcher, nPIST, and autophagy. Neuron. 35: 813-4. PMID 12372273 DOI: 10.1016/S0896-6273(02)00871-1 |
0.332 |
|
| 2002 |
Orr HT. Microarrays and polyglutamine disorders: reports from the Hereditary Disease Array Group. Human Molecular Genetics. 11: 1909-10. PMID 12165553 DOI: 10.1093/Hmg/11.17.1909 |
0.398 |
|
| 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 |
0.43 |
|
| 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 |
0.334 |
|
| 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 |
0.455 |
|
| 2001 |
Orr HT. Hereditary ataxia. An unfolded protein. Lancet. 358: S35. PMID 11784584 DOI: 10.1016/S0140-6736(01)07048-9 |
0.325 |
|
| 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 |
0.356 |
|
| 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 |
0.301 |
|
| 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 DOI: 10.1093/Hmg/10.20.2307 |
0.401 |
|
| 2001 |
Orr HT. Qs in the nucleus. Neuron. 31: 875-6. PMID 11580886 DOI: 10.1016/S0896-6273(01)00435-4 |
0.386 |
|
| 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 |
0.409 |
|
| 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 DOI: 10.1093/Hmg/10.14.1511 |
0.451 |
|
| 2001 |
Orr HT. Beyond the Qs in the polyglutamine diseases. Genes & Development. 15: 925-32. PMID 11316786 DOI: 10.1101/Gad.888401 |
0.305 |
|
| 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 DOI: 10.1093/Hmg/10.1.25 |
0.335 |
|
| 2001 |
Davidson JD, Orr HT. Expanding our understanding of polyglutamine disease through transgenic mice Current Genomics. 2: 27-39. DOI: 10.2174/1389202013351183 |
0.335 |
|
| 2000 |
Fernandez-Funez P, Nino-Rosales ML, de Gouyon B, She WC, Luchak JM, Martinez P, Turiegano E, Benito J, Capovilla M, Skinner PJ, McCall A, Canal I, Orr HT, Zoghbi HY, Botas J. Identification of genes that modify ataxin-1-induced neurodegeneration. Nature. 408: 101-6. PMID 11081516 DOI: 10.1038/35040584 |
0.447 |
|
| 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 DOI: 10.1093/Oxfordjournals.Hmg.A018922 |
0.763 |
|
| 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 |
0.426 |
|
| 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 |
0.339 |
|
| 2000 |
Orr HT, Zoghbi HY. Reversing neurodegeneration: a promise unfolds. Cell. 101: 1-4. PMID 10778849 DOI: 10.1016/S0092-8674(00)80617-0 |
0.421 |
|
| 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: 10.1093/jnen/59.4.265 |
0.353 |
|
| 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 DOI: 10.1093/Hmg/9.5.779 |
0.38 |
|
| 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 |
0.385 |
|
| 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 DOI: 10.1016/S0896-6273(00)81035-1 |
0.408 |
|
| 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 |
0.37 |
|
| 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 |
0.39 |
|
| 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 |
0.465 |
|
| 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 |
0.424 |
|
| 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 |
0.372 |
|
| 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 |
0.376 |
|
| 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 DOI: 10.1523/Jneurosci.18-14-05508.1998 |
0.369 |
|
| 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 |
0.436 |
|
| 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 |
0.384 |
|
| 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 |
0.306 |
|
| 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 DOI: 10.1523/Jneurosci.17-19-07385.1997 |
0.385 |
|
| 1997 |
Burright EN, Orr HT, Clark HB. Mouse models of human CAG repeat disorders. Brain Pathology (Zurich, Switzerland). 7: 965-77. PMID 9217978 DOI: 10.1111/J.1750-3639.1997.Tb00896.X |
0.401 |
|
| 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 |
0.352 |
|
| 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 |
0.35 |
|
| 1997 |
Clark HB, Burright EN, Yunis WS, Zoahbi HY, Orr HT. PURKINJE CELL HYPERTROPHY, A POSSIBLE COMPENSATORY MECHANISM IN SCA1-TRANSGENIC MICE Journal of Neuropathology and Experimental Neurology. 56: 587. DOI: 10.1097/00005072-199705000-00077 |
0.314 |
|
| 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 DOI: 10.1101/Sqb.1996.061.01.065 |
0.347 |
|
| 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 |
0.355 |
|
| 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 |
0.401 |
|
| 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 |
0.395 |
|
| 1996 |
Clark HB, Burright EN, Yunis WS, Fahssi WM, Zoghbi HY, Orr HT. CHARACTERIZATION OF TRANSGENIC MICE MADE ATAXIC BY EXPRESSION OF A MUTANT ALLELE FOR ATAXIN-1 THAT CONTAINS AN EXPANDED CAG REPEAT Journal of Neuropathology and Experimental Neurology. 55: 660. DOI: 10.1097/00005072-199605000-00228 |
0.355 |
|
| 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 |
0.331 |
|
| 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 |
0.398 |
|
| 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 |
0.379 |
|
| 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 |
0.402 |
|
| 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 |
0.351 |
|
| 1995 |
Clark HB, Orr HT, Yunis WS, Ebner TJ, Iadecola C, Feddersen RM. A MUTATION IN THE pRB-BINDING REGION OF SV40 T-ANTIGEN (Tag) INHIBITS TARGETED PURKINJE CELL DEATH IN TRANSGENIC MICE BUT DOES NOT PREVENT DEVELOPMENT OF ATAXIA Journal of Neuropathology and Experimental Neurology. 54: 460. DOI: 10.1097/00005072-199505000-00213 |
0.32 |
|
| 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 |
0.333 |
|
| 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 |
0.335 |
|
| 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 |
0.309 |
|
| 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 |
0.337 |
|
| 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 |
0.331 |
|
| 1993 |
Bonnycastle LL, Yu CE, Wijsman EM, Orr HT, Patterson D, Clancy KP, Goddard KA, Alonso ME, Nemens E, White JA. The c-fos gene and early-onset familial Alzheimer's disease. Neuroscience Letters. 160: 33-6. PMID 8247328 DOI: 10.1016/0304-3940(93)90910-D |
0.339 |
|
| 1993 |
Clark HB, Orr HT, Ehlenfeldt R, Yunis WS, Feddersen RM. PURKINJE CELL DEATH IN TRANSGENIC MICE EXPRESSING AN INTACT OR TRUNCATED FORM OF SV40 T ANTIGEN Journal of Neuropathology and Experimental Neurology. 52: 286. DOI: 10.1097/00005072-199305000-00105 |
0.311 |
|
| 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 DOI: 10.1212/Wnl.42.2.344 |
0.305 |
|
| 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 DOI: 10.1096/Fasebj.6.6.1544544 |
0.303 |
|
| 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 |
0.347 |
|
| 1992 |
Schellenberg GD, Bird TD, Wijsman EM, Orr HT, Anderson L, Nemens E, White JA, Bonnycastle L, Weber JL, Alonso ME. Genetic linkage evidence for a familial Alzheimer's disease locus on chromosome 14. Science (New York, N.Y.). 258: 668-71. PMID 1411576 DOI: 10.1126/Science.1411576 |
0.306 |
|
| 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 DOI: 10.1101/Gad.5.7.1136 |
0.357 |
|
| 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 |
0.353 |
|
| 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 |
0.304 |
|
| 1990 |
St George-Hyslop PH, Haines JL, Farrer LA, Polinsky R, Van Broeckhoven C, Goate A, McLachlan DR, Orr H, Bruni AC, Sorbi S, Rainero I, Foncin JF, Pollen D, Cantu JM, Tupler R, et al. Genetic linkage studies suggest that Alzheimer's disease is not a single homogeneous disorder. Nature. 347: 194-7. PMID 2395471 DOI: 10.1038/347194A0 |
0.343 |
|
| 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 DOI: 10.1084/Jem.171.1.1 |
0.548 |
|
| 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 DOI: 10.1084/Jem.169.2.469 |
0.544 |
|
| 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 DOI: 10.1073/Pnas.85.1.227 |
0.477 |
|
| 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 DOI: 10.1523/Jneurosci.08-12-04780.1988 |
0.37 |
|
| 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 DOI: 10.1073/Pnas.84.24.9145 |
0.541 |
|
| 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 |
0.499 |
|
| 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 DOI: 10.1073/Pnas.84.23.8535 |
0.447 |
|
| 1986 |
Shimizu Y, Koller B, Geraghty D, Orr H, Shaw S, Kavathas P, DeMars R. Transfer of cloned human class I major histocompatibility complex genes into HLA mutant human lymphoblastoid cells. Molecular and Cellular Biology. 6: 1074-1087. PMID 3023867 DOI: 10.1128/Mcb.6.4.1074 |
0.543 |
|
| 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 |
0.316 |
|
| 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 DOI: 10.1073/Pnas.81.16.5175 |
0.53 |
|
| 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 |
0.31 |
|
| 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 |
0.319 |
|
| 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 |
0.478 |
|
| 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 DOI: 10.1073/Pnas.79.12.3813 |
0.421 |
|
| 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 |
0.463 |
|
| 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 |
0.46 |
|
| 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 DOI: 10.1111/J.1365-3083.1980.Tb00026.X |
0.434 |
|
| 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 DOI: 10.1111/J.1365-3083.1980.Tb00025.X |
0.453 |
|
| 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 DOI: 10.1073/Pnas.77.10.6081 |
0.463 |
|
| 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 DOI: 10.1021/Bi00592A030 |
0.444 |
|
| 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 DOI: 10.1021/Bi00592A029 |
0.429 |
|
| 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 |
0.433 |
|
| 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 |
0.443 |
|
| 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 DOI: 10.1073/Pnas.76.9.4395 |
0.43 |
|
| 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 |
0.421 |
|
| 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 |
0.567 |
|
| 1976 |
Orr HT, Cohen AI, Lowry OH. The distribution of taurine in the vertebrate retina. Journal of Neurochemistry. 26: 609-11. PMID 816997 DOI: 10.1111/J.1471-4159.1976.Tb01519.X |
0.635 |
|
| 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 DOI: 10.1073/Pnas.73.12.4442 |
0.651 |
|
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