Research Output per year
Research Output 1972 2019
- 50 - 100 out of 250 results
- Publication Year, Title (descending)
SCA1-like disease in mice expressing wild-type Ataxin-1 with a serine to aspartic acid replacement at residue 776
Duvick, L., Barnes, J., Ebner, B., Agrawal, S., Andresen, M., Lim, J., Giesler, G. J., Zoghbi, H. Y. & Orr, H. T., Sep 1 2010, In : Neuron. 67, 6, p. 929-935 7 p.Research output: Contribution to journal › Article
Characterization of the zebrafish atxn1/axh gene family
Carlson, K. M., Melcher, L., Lai, S., Zoghbi, H. Y., Clark, H. B. & Orr, H. T., Jan 1 2009, In : Journal of Neurogenetics. 23, 3, p. 313-323 11 p.Research output: Contribution to journal › Article
Emerging pathogenic pathways in the spinocerebellar ataxias
Carlson, K. M., Andresen, J. M. & Orr, H. T., Jun 1 2009, In : Current Opinion in Genetics and Development. 19, 3, p. 247-253 7 p.Research output: Contribution to journal › Review article
Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, Spinocerebellar ataxia type 1
Zoghbi, H. Y. & Orr, H. T., Mar 20 2009, In : Journal of Biological Chemistry. 284, 12, p. 7425-7429 5 p.Research output: Contribution to journal › Short survey
Phosphorylation of ATXN1 at Ser776 in the cerebellum
Jorgensen, N. D., Andresen, J. M., Lagalwar, S., Armstrong, B., Stevens, S., Byam, C. E., Duvick, L. A., Lai, S., Jafar-Nejad, P., Zoghbi, H. Y., Clark, H. B. & Orr, H. T., Jul 1 2009, In : Journal of Neurochemistry. 110, 2, p. 675-686 12 p.Research output: Contribution to journal › Article
Unstable nucleotide repeat mini review series: A molecular biography of unstable repeat disorders
Orr, H. T., Mar 20 2009, In : Journal of Biological Chemistry. 284, 12, 1 p.Research output: Contribution to journal › Short survey
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
Gao, Y., Zu, T., Low, W. C., Orr, H. T. & McIvor, R. S., Dec 1 2008, In : Cell transplantation. 17, 7, p. 723-734 12 p.Research output: Contribution to journal › Article
miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis
Lee, Y., Samaco, R. C., Gatchel, J. R., Thaller, C., Orr, H. T. & Zoghbi, H. Y., Oct 1 2008, In : Nature neuroscience. 11, 10, p. 1137-1139 3 p.Research output: Contribution to journal › Article
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1
Lim, J., Crespo-Barreto, J., Jafar-Nejad, P., Bowman, A. B., Richman, R., Hill, D. E., Orr, H. T. & Zoghbi, H. Y., Apr 10 2008, In : Nature. 452, 7188, p. 713-718 6 p.Research output: Contribution to journal › Article
The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7
Gatchel, J. R., Watase, K., Thaller, C., Carson, J. P., Jafar-Nejad, P., Shaw, C., Zu, T., Orr, H. T. & Zoghbi, H. Y., Jan 29 2008, In : Proceedings of the National Academy of Sciences of the United States of America. 105, 4, p. 1291-1296 6 p.Research output: Contribution to journal › Article
Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes
Bowman, A. B., Lam, Y. C., Jafar-Nejad, P., Chen, H. K., Richman, R., Samaco, R. C., Fryer, J. D., Kahle, J. J., Orr, H. T. & Zoghbi, H. Y., Mar 1 2007, In : Nature Genetics. 39, 3, p. 373-379 7 p.Research output: Contribution to journal › Article
Fragile X Tremor/Ataxia Syndrome: Blame the Messenger!
Swanson, M. S. & Orr, H. T., Aug 16 2007, In : Neuron. 55, 4, p. 535-537 3 p.Research output: Contribution to journal › Short survey
Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin-1
Jorgensen, N. D., Andresen, J. M., Pitt, J. E., Swenson, M. A., Zoghbi, H. Y. & Orr, H. T., Sep 1 2007, In : Journal of Neurochemistry. 102, 6, p. 2040-2048 9 p.Research output: Contribution to journal › Article
Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model
Watase, K., Gatchel, J. R., Sun, Y., Emamian, E., Atkinson, R., Richman, R., Mizusawa, H., Orr, H. T., Shaw, C. & Zoghbi, H. Y., May 1 2007, In : PLoS medicine. 4, 5, p. 836-847 12 p.Research output: Contribution to journal › Article
Spinocerebellar ataxia type 1
Orr, H. T., Dec 1 2007, Neurobiology of Disease. Elsevier Inc., p. 149-155 7 p.Research output: Chapter in Book/Report/Conference proceeding › Chapter
Trinucleotide repeat disorders
Orr, H. T. & Zoghbi, H. Y., Aug 13 2007, In : Annual Review of Neuroscience. 30, p. 575-621 47 p.Research output: Contribution to journal › Review article
ATAXIN-1 Interacts with the Repressor Capicua in Its Native Complex to Cause SCA1 Neuropathology
Lam, Y. C., Bowman, A. B., Jafar-Nejad, P., Lim, J., Richman, R., Fryer, J. D., Hyun, E. D., Duvick, L. A., Orr, H. T., Botas, J. & Zoghbi, H. Y., Dec 29 2006, In : Cell. 127, 7, p. 1335-1347 13 p.Research output: Contribution to journal › Article
Neurodegenerative disease: Cut to the chase
Ellerby, L. M. & Orr, H. T., Aug 10 2006, In : Nature. 442, 7103, p. 641-642 2 p.Research output: Contribution to journal › Short survey
Phosphorylation Of Ataxin-1: A link between basic research and clinical application in spinocerebellar ataxia type 1
Carlson, K. M. & Orr, H. T., Dec 1 2006, Genetic Instabilities and Neurological Diseases, Second Edition. Elsevier Inc., p. 339-349 11 p.Research output: Chapter in Book/Report/Conference proceeding › Chapter
Polyglutamine neurodegenerative diseases and regulation of transcription: Assembling the puzzle
Riley, B. E. & Orr, H. T., Aug 15 2006, In : Genes and Development. 20, 16, p. 2183-2192 10 p.Research output: Contribution to journal › Review article
RORα-Mediated Purkinje Cell Development Determines Disease Severity in Adult SCA1 Mice
Serra, H. G., Duvick, L., Zu, T., Carlson, K., Stevens, S., Jorgensen, N., Lysholm, A., Burright, E., Zoghbi, H. Y., Clark, H. B., Andresen, J. M. & Orr, H. T., Nov 17 2006, In : Cell. 127, 4, p. 697-708 12 p.Research output: Contribution to journal › Article
Targeted deletion of a single Sca8 ataxia locus allele in mice causes abnormal gait, progressive loss of motor coordination, and Purkinje cell dendritic deficits
He, Y., Zu, T., Benzow, K. A., Orr, H. T., Clark, H. B. & Koob, M. D., Sep 27 2006, In : Journal of Neuroscience. 26, 39, p. 9975-9982 8 p.Research output: Contribution to journal › Article
The genetics of neurodegenerative diseases
Hardy, J. & Orr, H., Jun 1 2006, In : Journal of Neurochemistry. 97, 6, p. 1690-1699 10 p.Research output: Contribution to journal › Review article
A cell-based screen for modulators of ataxin-1 phosphorylation
Kaytor, M. D., Byam, C. E., Tousey, S. K., Stevens, S. D., Zoghbi, H. Y. & Orr, H. T., Apr 15 2005, In : Human molecular genetics. 14, 8, p. 1095-1105 11 p.Research output: Contribution to journal › Article
Animal models of spinocerebellar ataxia sype 1 (SCA1)
Kaytor, M. D. & Orr, H. T., Dec 1 2005, Movement Disorders. Elsevier Inc., p. 623-630 8 p.Research output: Chapter in Book/Report/Conference proceeding › Chapter
Identification of a novel phosphorylation site in ataxin-1
Vierra-Green, C. A., Orr, H. T., Zoghbi, H. Y. & Ferrington, D. A., May 15 2005, In : Biochimica et Biophysica Acta - Molecular Cell Research. 1744, 1, p. 11-18 8 p.Research output: Contribution to journal › Article
RNA association and nucleocytoplasmic shuttling by ataxin-1
Irwin, S., Vandelft, M., Pinchev, D., Howell, J. L., Graczyk, J., Orr, H. T. & Truant, R., Jan 1 2005, In : Journal of cell science. 118, 1, p. 233-242 10 p.Research output: Contribution to journal › Article
SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal
Riley, B. E., Zoghbi, H. Y. & Orr, H. T., Jun 10 2005, In : Journal of Biological Chemistry. 280, 23, p. 21942-21948 7 p.Research output: Contribution to journal › Article
The AXH domain of ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/senseless proteins
Tsuda, H., Jafar-Nejad, H., Patel, A. J., Sun, Y., Chen, H. K., Rose, M. F., Venken, K. J. T., Botas, J., Orr, H. T., Bellen, H. J. & Zoghbi, H. Y., Aug 16 2005, In : Cell. 122, 4, p. 633-644 12 p.Research output: Contribution to journal › Article
Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice
Serra, H. G., Byam, C. E., Lande, J. D., Tousey, S. K., Zoghbi, H. Y. & Orr, H. T., Oct 15 2004, In : Human molecular genetics. 13, 20, p. 2535-2543 9 p.Research output: Contribution to journal › Article
Generation and Characterization of LANP/pp32 Null Mice
Opal, P., Garcia, J. J., McCall, A. E., Xu, B., Weeber, E. J., Sweatt, J. D., Orr, H. T. & Zoghbi, H. Y., Apr 1 2004, In : Molecular and cellular biology. 24, 8, p. 3140-3149 10 p.Research output: Contribution to journal › Article
Into the depths of ataxia
Orr, H. T., Feb 1 2004, In : Journal of Clinical Investigation. 113, 4, p. 505-507 3 p.Research output: Contribution to journal › Review article
Neuron protection agency
Orr, H. T., Oct 14 2004, In : Nature. 431, 7010, p. 747-748 2 p.Research output: Contribution to journal › Short survey
Overexpression of CREB reduces CRE-mediated transcription: Behavioral and cellular analyses in transgenic mice
Brodie, C. R., Khaliq, M., Yin, J. C. P., Clark, H. B., Orr, H. T. & Boland, L. M., Apr 1 2004, In : Molecular and Cellular Neuroscience. 25, 4, p. 602-611 10 p.Research output: Contribution to journal › Article
Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice
Zu, T., Duvick, L. A., Kaytor, M. D., Berlinger, M. S., Zoghbi, H. Y., Clark, H. B. & Orr, H. T., Oct 6 2004, In : Journal of Neuroscience. 24, 40, p. 8853-8861 9 p.Research output: Contribution to journal › Article
RNA gains a new function: A mediator of neurodegeneration
Orr, H. T., May 1 2004, In : Trends in Neurosciences. 27, 5, p. 233-234 2 p.Research output: Contribution to journal › Short survey
RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia
Xia, H., Mao, Q., Eliason, S. L., Harper, S. Q., Martins, I. H., Orr, H. T., Paulson, H. L., Yang, L., Kotin, R. M. & Davidson, B. L., Aug 1 2004, In : Nature Medicine. 10, 8, p. 816-820 5 p.Research output: Contribution to journal › Article
The effects of the polyglutamine repeat protein ataxin-1 on the UbL-UBA protein A1UP
Riley, B. E., Xu, Y., Zoghbi, H. Y. & Orr, H. T., Oct 1 2004, In : Journal of Biological Chemistry. 279, 40, p. 42290-42301 12 p.Research output: Contribution to journal › Article
Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1
Chen, H. K., Fernandez-Funez, P., Acevedo, S. F., Lam, Y. C., Kaytor, M. D., Fernandez, M. H., Aitken, A., Skoulakis, E. M. C., Orr, H. T., Botas, J. & Zoghbi, H. Y., May 16 2003, In : Cell. 113, 4, p. 457-468 12 p.Research output: Contribution to journal › Article
Mapmodulin/Leucine-rich Acidic Nuclear Protein Binds the Light Chain of Microtubule-associated Protein 1B and Modulates Neuritogenesis
Opal, P., Garcia, J. J., Propst, F., Matilla, A., Orr, H. T. & Zoghbi, H. Y., Sep 5 2003, In : Journal of Biological Chemistry. 278, 36, p. 34691-34699 9 p.Research output: Contribution to journal › Article
Regional differences of somatic CAG repeat instability do not account for selective neuronal vulnerability in a knock-in mouse model of SCA1
Watase, K., Venken, K. J. T., Sun, Y., Orr, H. T. & Zoghbi, H. Y., Nov 1 2003, In : Human molecular genetics. 12, 21, p. 2789-2795 7 p.Research output: Contribution to journal › Article
Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice
Emamian, E. S., Kaytor, M. D., Duvick, L. A., Zu, T., Tousey, S. K., Zoghbi, H. Y., Clark, H. B. & Orr, H. T., May 8 2003, In : Neuron. 38, 3, p. 375-387 13 p.Research output: Contribution to journal › Article
Spinocerebellar Ataxia 1 (SCA1)
Orr, H. T., Jan 1 2003, Genetics of Movement Disorders. Elsevier Inc., p. 35-43 9 p.Research output: Chapter in Book/Report/Conference proceeding › Chapter
A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration
Watase, K., Weeber, E. J., Xu, B., Antalffy, B., Yuva-Paylor, L., Hashimoto, K., Kano, M., Atkinson, R., Sun, Y., Armstrong, D. L., Sweatt, J. D., Orr, H. T., Paylor, R. & Zoghbi, H. Y., Jun 13 2002, In : Neuron. 34, 6, p. 905-919 15 p.Research output: Contribution to journal › Article
Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice
Skinner, P. J., Vierra-Green, C. A., Emamian, E., Zoghbi, H. Y. & Orr, H. T., Dec 1 2002, In : NeuroMolecular Medicine. 1, 1, p. 33-42 10 p.Research output: Contribution to journal › Article
A proposed mechanism of ALS fails the test in vivo
Orr, H. T., Apr 16 2002, In : Nature neuroscience. 5, 4, p. 287-288 2 p.Research output: Contribution to journal › Short survey
Lurcher, nPIST, and autophagy
Orr, H. T., Aug 29 2002, In : Neuron. 35, 5, p. 813-814 2 p.Research output: Contribution to journal › Review article
Microarrays and polyglutamine disorders: Reports from the hereditary disease array group
Orr, H. T., Aug 15 2002, In : Human molecular genetics. 11, 17, p. 1909-1910 2 p.Research output: Contribution to journal › Article
The GSK3β signaling cascade and neurodegenerative disease
Kaytor, M. D. & Orr, H. T., Jun 1 2002, In : Current opinion in neurobiology. 12, 3, p. 275-278 4 p.Research output: Contribution to journal › Review article
Altered trafficking of membrane proteins in Purkinje cells of SCA1 transgenic mice
Skinner, P. J., Vierra-Green, C. A., Clark, H. B., Zoghbi, H. Y. & Orr, H. T., Jan 1 2001, In : American Journal of Pathology. 159, 3, p. 905-913 9 p.Research output: Contribution to journal › Article