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Research Output 1972 2019

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2019

Treadmill training increases the motor activity and neuron survival of the cerebellum in a mouse model of spinocerebellar ataxia type 1

Chuang, C. S., Chang, J. C., Soong, B. W., Chuang, S. F., Lin, T. T., Cheng, W. L., Orr, H. T. & Liu, C. S., Jan 1 2019, In : Kaohsiung Journal of Medical Sciences.

Research output: Contribution to journalArticle

Open Access
Spinocerebellar Ataxias
Motor Neurons
Cerebellum
Motor Activity
Exercise
2018
4 Citations (Scopus)

Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles

Friedrich, J., Kordasiewicz, H. B., O'Callaghan, B., Handler, H. P., Wagener, C., Duvick, L., Swayze, E. E., Rainwater, O., Hofstra, B., Benneyworth, M., Nichols-Meade, T., Yang, P., Chen, Z., Ortiz, J. P., Clark, H. B., Oz, G., Larson, S., Zoghbi, H. Y., Henzler, C. & Orr, H. T., Nov 2 2018, In : JCI Insight. 3, 21

Research output: Contribution to journalArticle

Open Access
Spinocerebellar Ataxias
Antisense Oligonucleotides
Transcriptome
Neurological Models
RNA
6 Citations (Scopus)

ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism

Rousseaux, M. W. C., Tschumperlin, T., Lu, H. C., Lackey, E. P., Bondar, V. V., Wan, Y. W., Tan, Q., Adamski, C. J., Friedrich, J., Twaroski, K., Chen, W., Tolar, J., Henzler, C., Sharma, A., Bajić, A., Lin, T., Duvick, L., Liu, Z., Sillitoe, R. V., Zoghbi, H. Y. & 1 others, Orr, H. T., Mar 21 2018, In : Neuron. 97, 6, p. 1235-1243.e5

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Pathology
Cerebellum
Neurons
Purkinje Cells
3 Citations (Scopus)

Motor neuron degeneration correlates with respiratory dysfunction in SCA1

Orengo, J. P., van der Heijden, M. E., Hao, S., Tang, J., Orr, H. T. & Zoghbi, H. Y., Feb 26 2018, In : Disease models & mechanisms. 11, 2

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Nerve Degeneration
Motor Neurons
Neurons
Diaphragm
2 Citations (Scopus)

PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1

Bondar, V. V., Adamski, C. J., Onur, T. S., Tan, Q., Wang, L., Diaz-Garcia, J., Park, J., Orr, H. T., Botas, J. & Zoghbi, H. Y., Jan 1 2018, In : Human molecular genetics. 27, 16, p. 2863-2873 11 p.

Research output: Contribution to journalArticle

Open Access
Spinocerebellar Ataxias
p21-Activated Kinases
Pharmacology
Ataxin-1
Drosophila
2 Citations (Scopus)
Spinocerebellar Ataxias
Optical Coherence Tomography
Atrophy
Neurodegenerative Diseases
Birefringence
1 Citation (Scopus)

RBM17 Interacts with U2SURP and CHERP to Regulate Expression and Splicing of RNA-Processing Proteins

De Maio, A., Yalamanchili, H. K., Adamski, C. J., Gennarino, V. A., Liu, Z., Qin, J., Jung, S. Y., Richman, R., Orr, H. T. & Zoghbi, H. Y., Oct 16 2018, In : Cell reports. 25, 3, p. 726-736.e7

Research output: Contribution to journalArticle

Open Access
RNA Splicing
RNA-Binding Proteins
Spliceosomes
RNA
Processing
1 Citation (Scopus)

Reduction of protein kinase A-mediated phosphorylation of ATXN1-S776 in Purkinje cells delays onset of Ataxia in a SCA1 mouse model

Pérez Ortiz, J. M., Mollema, N., Toker, N., Adamski, C. J., O'Callaghan, B., Duvick, L., Friedrich, J., Walters, M. A., Strasser, J. M., Hawkinson, J. E., Zoghbi, H. Y., Henzler, C., Orr, H. T. & Lagalwar, S., Aug 1 2018, In : Neurobiology of Disease. 116, p. 93-105 13 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Purkinje Cells
Ataxia
Cyclic AMP-Dependent Protein Kinases
Phosphorylation
2017
23 Citations (Scopus)

Disruption of the ATXN1-CIC complex causes a spectrum of neurobehavioral phenotypes in mice and humans

Lu, H. C., Tan, Q., Rousseaux, M. W. C., Wang, W., Kim, J. Y., Richman, R., Wan, Y. W., Yeh, S. Y., Patel, J. M., Liu, X., Lin, T., Lee, Y., Fryer, J. D., Han, J., Chahrour, M., Finnell, R. H., Lei, Y., Zurita-Jimenez, M. E., Ahimaz, P., Anyane-Yeboa, K. & 17 others, Van Maldergem, L., Lehalle, D., Jean-Marcais, N., Mosca-Boidron, A. L., Thevenon, J., Cousin, M. A., Bro, D. E., Lanpher, B. C., Klee, E. W., Alexander, N., Bainbridge, M. N., Orr, H. T., Sillitoe, R. V., Ljungberg, M. C., Liu, Z., Schaaf, C. P. & Zoghbi, H. Y., Mar 30 2017, In : Nature Genetics. 49, 4, p. 527-536 10 p.

Research output: Contribution to journalArticle

Open Access
Phenotype
Mutation
Middle Hypothalamus
Attention Deficit Disorder with Hyperactivity
Interpersonal Relations
8 Citations (Scopus)

Visualizing and mapping the cerebellum with serial optical coherence scanner

Liu, C. J., Williams, K. E., Orr, H. T. & Akkin, T., Jan 1 2017, In : Neurophotonics. 4, 1, 011006.

Research output: Contribution to journalArticle

Open Access
Cerebellum
Brain Stem
Optical Coherence Tomography
Immersion
Nerve Fibers
2016
31 Citations (Scopus)

Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways

Ingram, M., Wozniak, E. A. L., Duvick, L., Yang, R., Bergmann, P., Carson, R., O'Callaghan, B., Zoghbi, H. Y., Henzler, C. & Orr, H. T., Mar 16 2016, In : Neuron. 89, 6, p. 1194-1207 14 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Purkinje Cells
Transcriptome
Transgenic Mice
Disease Progression
8 Citations (Scopus)
Neurodegenerative diseases
Neurodegenerative Diseases
Huntington Disease
Spinocerebellar Ataxias
Therapeutics
29 Citations (Scopus)

Extensive cryptic splicing upon loss of RBM17 and TDP43 in neurodegeneration models

Tan, Q., Yalamanchili, H. K., Park, J., De Maio, A., Lu, H. C., Wan, Y. W., White, J. J., Bondar, V. V., Sayegh, L. S., Liu, X., Gao, Y., Sillitoe, R. V., Orr, H. T., Liu, Z. & Zoghbi, H. Y., Jan 1 2016, In : Human molecular genetics. 25, 23, p. 5083-5093 11 p.

Research output: Contribution to journalArticle

RNA-Binding Proteins
Cell Survival
Protein Splicing
Genes
Survival
65 Citations (Scopus)

Tolerance is established in polyclonal CD4 + T cells by distinct mechanisms, according to self-peptide expression patterns

Malhotra, D., Linehan, J. L., Dileepan, T., Lee, Y. J., Purtha, W. E., Lu, J. V., Nelson, R. W., Fife, B. T., Orr, H. T., Anderson, M. S., Hogquist, K. A. & Jenkins, M. K., Feb 1 2016, In : Nature immunology. 17, 2, p. 187-195 9 p.

Research output: Contribution to journalArticle

T-Lymphocytes
Peptides
Clonal Deletion
Self Tolerance
T-Lymphocyte Epitopes
2015
14 Citations (Scopus)

A native interactor scaffolds and stabilizes toxic Ataxin-1 oligomers in SCA1

Lasagna-Reeves, C. A., Rousseaux, M. W. C., Guerrero-Munoz, M. J., Park, J., Jafar-Nejad, P., Richman, R., Lu, N., Sengupta, U., Litvinchuk, A., Orr, H. T., Kayed, R. & Zoghbi, H. Y., May 19 2015, In : eLife. 4, MAY, p. 1-46 46 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Poisons
Oligomers
Scaffolds
Brain
7 Citations (Scopus)
Spinocerebellar Ataxias
Transgenes
Histology
Magnetic Resonance Spectroscopy
Gene Expression
9 Citations (Scopus)

Ataxin-1 oligomers induce local spread of pathology and decreasing them by passive immunization slows spinocerebellar ataxia type 1 phenotypes

Lasagna-Reeves, C. A., Rousseaux, M. W. C., Guerrero-Munoz, M. J., Vilanova-Velez, L., Park, J., See, L., Jafar-Nejad, P., Richman, R., Orr, H. T., Kayed, R. & Zoghbi, H. Y., Dec 17 2015, In : eLife. 4, DECEMBER2015, e10891.

Research output: Contribution to journalArticle

Immunization
Spinocerebellar Ataxias
Passive Immunization
Pathology
Oligomers
24 Citations (Scopus)

Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1

Cvetanovic, M., Ingram, M., Orr, H. T. & Opal, P., Mar 9 2015, In : Neuroscience. 289, p. 289-299 11 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Microglia
Neuroglia
Astrocytes
Disease Progression
42 Citations (Scopus)

Neuronal atrophy early in degenerative ataxia is a compensatory mechanism to regulate membrane excitability

Dell’Orco, J. M., Wasserman, A. H., Chopra, R., Ingram, M. A. C., Hu, Y. S., Singh, V., Wulff, H., Opal, P., Orr, H. T. & Shakkottai, V. G., Aug 12 2015, In : Journal of Neuroscience. 35, 32, p. 11292-11307 16 p.

Research output: Contribution to journalArticle

Purkinje Cells
Ataxia
Atrophy
Spinocerebellar Ataxias
Membranes
48 Citations (Scopus)

Pumilio1 haploinsufficiency leads to SCA1-like neurodegeneration by increasing wild-type Ataxin1 levels

Gennarino, V. A., Singh, R. K., White, J. J., De Maio, A., Han, K., Kim, J. Y., Jafar-Nejad, P., Di Ronza, A., Kang, H., Sayegh, L. S., Cooper, T. A., Orr, H. T., Sillitoe, R. V. & Zoghbi, H. Y., Mar 15 2015, In : Cell. 160, 6, p. 1087-1098 12 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Haploinsufficiency
Neurodegenerative diseases
Breeding
RNA-Binding Proteins
2013

One family's search to explain a fatal neurological disorder

Mollema, N. J. & Orr, H. T., Jan 1 2013, American Scientist, 101, 6, p. 442-449 8 p.

Research output: Contribution to specialist publicationArticle

Ataxia
Nervous System Diseases
Fathers
Aptitude
Shoes
10 Citations (Scopus)

Polyglutamine disease toxicity is regulated by nemo-like kinase in spinocerebellar ataxia type 1

Ju, H., Kokubu, H., Todd, T. W., Kahle, J. J., Kim, S., Richman, R., Chirala, K., Orr, H. T., Zoghbi, H. Y. & Lim, J., May 29 2013, In : Journal of Neuroscience. 33, 22, p. 9328-9336 9 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Phosphotransferases
Phenotype
Trinucleotide Repeats
Protein-Serine-Threonine Kinases
27 Citations (Scopus)

Purkinje cell ataxin-1 Modulates climbing fiber synaptic input in developing and adult mouse cerebellum

Ebner, B. A., Ingram, M. A., Barnes, J. A., Duvick, L. A., Frisch, J. L., Clark, H. B., Zoghbi, H. Y., Ebner, T. J. & Orr, H. T., Mar 27 2013, In : Journal of Neuroscience. 33, 13, p. 5806-5820 15 p.

Research output: Contribution to journalArticle

Purkinje Cells
Cerebellum
Ataxin-1
Transgenic Mice
Spinocerebellar Ataxias
74 Citations (Scopus)

RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1

Park, J., Al-Ramahi, I., Tan, Q., Mollema, N., Diaz-Garcia, J. R., Gallego-Flores, T., Lu, H. C., Lagalwar, S., Duvick, L., Kang, H., Lee, Y., Jafar-Nejad, P., Sayegh, L. S., Richman, R., Liu, X., Gao, Y., Shaw, C. A., Arthur, J. S. C., Orr, H. T., Westbrook, T. F. & 2 others, Botas, J. & Zoghbi, H. Y., May 30 2013, In : Nature. 498, 7454, p. 325-331 7 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Neurodegenerative Diseases
Drosophila
Proteins
Genetic Testing
2011
35 Citations (Scopus)

14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus

Lai, S., O'Callaghan, B., Zoghbi, H. Y. & Orr, H. T., Oct 7 2011, In : Journal of Biological Chemistry. 286, 40, p. 34606-34616 11 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Phosphorylation
Ataxin-1
Purkinje Cells
Pathology
47 Citations (Scopus)

Abnormalities in the climbing fiber-Purkinje cell circuitry contribute to neuronal dysfunction in ATXN1[82Q] mice

Barnes, J. A., Ebner, B. A., Duvick, L. A., Gao, W., Chen, G., Orr, H. T. & Ebner, T. J., Sep 7 2011, In : Journal of Neuroscience. 31, 36, p. 12778-12789 12 p.

Research output: Contribution to journalArticle

Purkinje Cells
Optical Imaging
Synaptic Transmission
Vesicular Glutamate Transport Protein 2
Flavoproteins
76 Citations (Scopus)

Aminopyridines correct early dysfunction and delay neurodegeneration in a mouse model of spinocerebellar ataxia type

Hourez, R., Servais, L., Orduz, D., Gall, D., Millard, I., de Kerchove d'Exaerde, A., Cheron, G., Orr, H. T., Pandolfo, M. & Schiffmann, S. N., Aug 17 2011, In : Journal of Neuroscience. 31, 33, p. 11795-11807 13 p.

Research output: Contribution to journalArticle

Aminopyridines
Spinocerebellar Ataxias
Purkinje Cells
Atrophy
Animal Behavior
44 Citations (Scopus)

ATXN1 Protein Family and CIC Regulate Extracellular Matrix Remodeling and Lung Alveolarization

Lee, Y., Fryer, J. D., Kang, H., Crespo-Barreto, J., Bowman, A. B., Gao, Y., Kahle, J. J., Hong, J. S., Kheradmand, F., Orr, H. T., Finegold, M. J. & Zoghbi, H. Y., Oct 18 2011, In : Developmental Cell. 21, 4, p. 746-757 12 p.

Research output: Contribution to journalArticle

Matrix Metalloproteinases
Extracellular Matrix
Genes
Lung
Defects
86 Citations (Scopus)

Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua

Fryer, J. D., Yu, P., Kang, H., Mandel-Brehm, C., Carter, A. N., Crespo-Barreto, J., Gao, Y., Flora, A., Shaw, C., Orr, H. T. & Zoghbi, H. Y., Nov 4 2011, In : Science. 334, 6056, p. 690-693 4 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Neurodegenerative Diseases
Phenotype
Poisons
Ataxia
18 Citations (Scopus)

In vivo monitoring of recovery from neurodegeneration in conditional transgenic SCA1 mice

Öz, G., Vollmers, M. L., Nelson, C. D., Shanley, R., Eberly, L. E., Orr, H. T. & Clark, H. B., Dec 1 2011, In : Experimental Neurology. 232, 2, p. 290-298 9 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
Inositol
Doxycycline
Taurine
Pathology
35 Citations (Scopus)

Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model

Gehrking, K. M., Andresen, J. M., Duvick, L. A., Lough, J., Zoghbi, H. Y. & Orr, H. T., Jun 1 2011, In : Human molecular genetics. 20, 11, p. 2204-2212 9 p., ddr108.

Research output: Contribution to journalArticle

tat Gene Products
Spinocerebellar Ataxias
Disease Progression
HMGB Proteins
Ataxin-1
39 Citations (Scopus)
Spinocerebellar Ataxias
Haploinsufficiency
Virulence
Phenotype
14-3-3 Proteins
2010
54 Citations (Scopus)
Spinocerebellar Ataxias
Magnetic Resonance Spectroscopy
Inositol
Transgenic Mice
Disease Progression
76 Citations (Scopus)

Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis

Crespo-Barreto, J., Fryer, J. D., Shaw, C. A., Orr, H. T. & Zoghbi, H. Y., Jul 2010, In : PLoS Genetics. 6, 7, p. 1-17 17 p.

Research output: Contribution to journalArticle

Spinocerebellar Ataxias
pathogenesis
neurodegenerative diseases
gene expression
disease models
97 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Aspartic Acid
Serine
Cell Death
Glutamine
2009
7 Citations (Scopus)

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 journalArticle

Zebrafish
Cerebellum
Genes
Spinocerebellar Ataxias
Vertebrates
30 Citations (Scopus)

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 journalArticle

Phosphorylation
Cerebellum
Spinocerebellar Ataxias
Inbred AKR Mouse
Thymoma
2008
7 Citations (Scopus)
Spinocerebellar Ataxias
Trinucleotide Repeats
Antisense RNA
Gene therapy
Molecular Models
163 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Neurodegenerative Diseases
Proteins
polyglutamine
223 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Proteins
Glutamine
Poisons
Mutant Proteins
50 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Somatomedins
Insulin-Like Growth Factor Binding Protein 5
Purkinje Cells
Glutamine
2007
53 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Glutamine
Neurodegenerative Diseases
Ships
Neuropathology
9 Citations (Scopus)

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 journalArticle

Phosphorylation
Spinocerebellar Ataxias
Cytoplasm
Serine
Cricetulus
119 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Neuronal Plasticity
Lithium
Lithium Carbonate
Therapeutics
2006
195 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Proteins
Neurodegenerative diseases
Neurodegenerative Diseases
Cerebellum
140 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Purkinje Cells
Neurodegenerative diseases
Gene expression
Transcription Factors
54 Citations (Scopus)
Purkinje Cells
Ataxia
Gait
Alleles
Antisense RNA
2005
23 Citations (Scopus)

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 journalArticle

Phosphorylation
Serine
Spinocerebellar Ataxias
Ataxin-1
Phosphotransferases
13 Citations (Scopus)

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 journalArticle

Spinocerebellar Ataxias
Serine
Phosphorylation
Alanine
Transgenic Mice
77 Citations (Scopus)

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 journalArticle

RNA
Spinocerebellar Ataxias
Intranuclear Inclusion Bodies
Cell Nucleus Active Transport
Nuclear RNA