A Servadio, B Koshy, D Armstrong, B Antalffy, H T Orr, H Y Zoghbi. Nat Genet 1995
Times Cited: 242
Times Cited: 242
Times Cited
Times Co-cited
Similarity
Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1.
H T Orr, M Y Chung, S Banfi, T J Kwiatkowski, A Servadio, A L Beaudet, A E McCall, L A Duvick, L P Ranum, H Y Zoghbi. Nat Genet 1993
H T Orr, M Y Chung, S Banfi, T J Kwiatkowski, A Servadio, A L Beaudet, A E McCall, L A Duvick, L P Ranum, H Y Zoghbi. Nat Genet 1993
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Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.
I A Klement, P J Skinner, M D Kaytor, H Yi, S M Hersch, H B Clark, H Y Zoghbi, H T Orr. Cell 1998
I A Klement, P J Skinner, M D Kaytor, H Yi, S M Hersch, H B Clark, H Y Zoghbi, H T Orr. Cell 1998
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SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat.
E N Burright, H B Clark, A Servadio, T Matilla, R M Feddersen, W S Yunis, L A Duvick, H Y Zoghbi, H T Orr. Cell 1995
E N Burright, H B Clark, A Servadio, T Matilla, R M Feddersen, W S Yunis, L A Duvick, H Y Zoghbi, H T Orr. Cell 1995
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ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology.
Yung C Lam, Aaron B Bowman, Paymaan Jafar-Nejad, Janghoo Lim, Ronald Richman, John D Fryer, Eric D Hyun, Lisa A Duvick, Harry T Orr, Juan Botas,[...]. Cell 2006
Yung C Lam, Aaron B Bowman, Paymaan Jafar-Nejad, Janghoo Lim, Ronald Richman, John D Fryer, Eric D Hyun, Lisa A Duvick, Harry T Orr, Juan Botas,[...]. Cell 2006
31
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.
Janghoo Lim, Juan Crespo-Barreto, Paymaan Jafar-Nejad, Aaron B Bowman, Ronald Richman, David E Hill, Harry T Orr, Huda Y Zoghbi. Nature 2008
Janghoo Lim, Juan Crespo-Barreto, Paymaan Jafar-Nejad, Aaron B Bowman, Ronald Richman, David E Hill, Harry T Orr, Huda Y Zoghbi. Nature 2008
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Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.
Hung-Kai Chen, Pedro Fernandez-Funez, Summer F Acevedo, Yung C Lam, Michael D Kaytor, Michael H Fernandez, Alastair Aitken, Efthimios M C Skoulakis, Harry T Orr, Juan Botas,[...]. Cell 2003
Hung-Kai Chen, Pedro Fernandez-Funez, Summer F Acevedo, Yung C Lam, Michael D Kaytor, Michael H Fernandez, Alastair Aitken, Efthimios M C Skoulakis, Harry T Orr, Juan Botas,[...]. Cell 2003
29
Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice.
Effat S Emamian, Michael D Kaytor, Lisa A Duvick, Tao Zu, Susan K Tousey, Huda Y Zoghbi, H Brent Clark, Harry T Orr. Neuron 2003
Effat S Emamian, Michael D Kaytor, Lisa A Duvick, Tao Zu, Susan K Tousey, Huda Y Zoghbi, H Brent Clark, Harry T Orr. Neuron 2003
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A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.
Kei Watase, Edwin J Weeber, Bisong Xu, Barbara Antalffy, Lisa Yuva-Paylor, Kouichi Hashimoto, Masanobu Kano, Richard Atkinson, Yaling Sun, Dawna L Armstrong,[...]. Neuron 2002
Kei Watase, Edwin J Weeber, Bisong Xu, Barbara Antalffy, Lisa Yuva-Paylor, Kouichi Hashimoto, Masanobu Kano, Richard Atkinson, Yaling Sun, Dawna L Armstrong,[...]. Neuron 2002
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Identification and characterization of the gene causing type 1 spinocerebellar ataxia.
S Banfi, A Servadio, M Y Chung, T J Kwiatkowski, A E McCall, L A Duvick, Y Shen, E J Roth, H T Orr, H Y Zoghbi. Nat Genet 1994
S Banfi, A Servadio, M Y Chung, T J Kwiatkowski, A E McCall, L A Duvick, Y Shen, E J Roth, H T Orr, H Y Zoghbi. Nat Genet 1994
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Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures.
P J Skinner, B T Koshy, C J Cummings, I A Klement, K Helin, A Servadio, H Y Zoghbi, H T Orr. Nature 1997
P J Skinner, B T Koshy, C J Cummings, I A Klement, K Helin, A Servadio, H Y Zoghbi, H T Orr. Nature 1997
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Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.
C J Cummings, M A Mancini, B Antalffy, D B DeFranco, H T Orr, H Y Zoghbi. Nat Genet 1998
C J Cummings, M A Mancini, B Antalffy, D B DeFranco, H T Orr, H Y Zoghbi. Nat Genet 1998
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Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation.
A Matilla, E D Roberson, S Banfi, J Morales, D L Armstrong, E N Burright, H T Orr, J D Sweatt, H Y Zoghbi, M M Matzuk. J Neurosci 1998
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Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice.
Tao Zu, Lisa A Duvick, Michael D Kaytor, Michael S Berlinger, Huda Y Zoghbi, H Brent Clark, Harry T Orr. J Neurosci 2004
Tao Zu, Lisa A Duvick, Michael D Kaytor, Michael S Berlinger, Huda Y Zoghbi, H Brent Clark, Harry T Orr. J Neurosci 2004
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RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice.
Heliane G Serra, Lisa Duvick, Tao Zu, Kerri Carlson, Sam Stevens, Nathan Jorgensen, Alana Lysholm, Eric Burright, Huda Y Zoghbi, H Brent Clark,[...]. Cell 2006
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The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract.
S Yue, H G Serra, H Y Zoghbi, H T Orr. Hum Mol Genet 2001
S Yue, H G Serra, H Y Zoghbi, H T Orr. Hum Mol Genet 2001
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The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins.
Hiroshi Tsuda, Hamed Jafar-Nejad, Akash J Patel, Yaling Sun, Hung-Kai Chen, Matthew F Rose, Koen J T Venken, Juan Botas, Harry T Orr, Hugo J Bellen,[...]. Cell 2005
Hiroshi Tsuda, Hamed Jafar-Nejad, Akash J Patel, Yaling Sun, Hung-Kai Chen, Matthew F Rose, Koen J T Venken, Juan Botas, Harry T Orr, Hugo J Bellen,[...]. Cell 2005
21
Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors.
Chih-Cheng Tsai, Hung-Ying Kao, Akifumi Mitzutani, Ester Banayo, Harini Rajan, Michael McKeown, Ronald M Evans. Proc Natl Acad Sci U S A 2004
Chih-Cheng Tsai, Hung-Ying Kao, Akifumi Mitzutani, Ester Banayo, Harini Rajan, Michael McKeown, Ronald M Evans. Proc Natl Acad Sci U S A 2004
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Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice.
Heliane G Serra, Courtney E Byam, Jeffrey D Lande, Susan K Tousey, Huda Y Zoghbi, Harry T Orr. Hum Mol Genet 2004
Heliane G Serra, Courtney E Byam, Jeffrey D Lande, Susan K Tousey, Huda Y Zoghbi, Harry T Orr. Hum Mol Genet 2004
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RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.
Haibin Xia, Qinwen Mao, Steven L Eliason, Scott Q Harper, Inês H Martins, Harry T Orr, Henry L Paulson, Linda Yang, Robert M Kotin, Beverly L Davidson. Nat Med 2004
Haibin Xia, Qinwen Mao, Steven L Eliason, Scott Q Harper, Inês H Martins, Harry T Orr, Henry L Paulson, Linda Yang, Robert M Kotin, Beverly L Davidson. Nat Med 2004
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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.
H B Clark, E N Burright, W S Yunis, S Larson, C Wilcox, B Hartman, A Matilla, H Y Zoghbi, H T Orr. J Neurosci 1997
H B Clark, E N Burright, W S Yunis, S Larson, C Wilcox, B Hartman, A Matilla, H Y Zoghbi, H T Orr. J Neurosci 1997
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Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.
C J Cummings, E Reinstein, Y Sun, B Antalffy, Y Jiang, A Ciechanover, H T Orr, A L Beaudet, H Y Zoghbi. Neuron 1999
C J Cummings, E Reinstein, Y Sun, B Antalffy, Y Jiang, A Ciechanover, H T Orr, A L Beaudet, H Y Zoghbi. Neuron 1999
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SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776.
Lisa Duvick, Justin Barnes, Blake Ebner, Smita Agrawal, Michael Andresen, Janghoo Lim, Glenn J Giesler, Huda Y Zoghbi, Harry T Orr. Neuron 2010
Lisa Duvick, Justin Barnes, Blake Ebner, Smita Agrawal, Michael Andresen, Janghoo Lim, Glenn J Giesler, Huda Y Zoghbi, Harry T Orr. Neuron 2010
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Identification of genes that modify ataxin-1-induced neurodegeneration.
P Fernandez-Funez, M L Nino-Rosales, B de Gouyon, W C She, J M Luchak, P Martinez, E Turiegano, J Benito, M Capovilla, P J Skinner,[...]. Nature 2000
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18
Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1.
X Lin, B Antalffy, D Kang, H T Orr, H Y Zoghbi. Nat Neurosci 2000
X Lin, B Antalffy, D Kang, H T Orr, H Y Zoghbi. Nat Neurosci 2000
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Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel.
O Zhuchenko, J Bailey, P Bonnen, T Ashizawa, D W Stockton, C Amos, W B Dobyns, S H Subramony, H Y Zoghbi, C C Lee. Nat Genet 1997
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Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1.
Huda Y Zoghbi, Harry T Orr. J Biol Chem 2009
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RNA association and nucleocytoplasmic shuttling by ataxin-1.
Stuart Irwin, Mark Vandelft, Deborah Pinchev, Jenny L Howell, Joanna Graczyk, Harry T Orr, Ray Truant. J Cell Sci 2005
Stuart Irwin, Mark Vandelft, Deborah Pinchev, Jenny L Howell, Joanna Graczyk, Harry T Orr, Ray Truant. J Cell Sci 2005
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Structural and immunocytochemical features of olivopontocerebellar atrophy caused by the spinocerebellar ataxia type 1 (SCA-1) mutation define a unique phenotype.
Y Robitaille, L Schut, S J Kish. Acta Neuropathol 1995
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Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1.
Akifumi Mizutani, Lei Wang, Harini Rajan, Parminder J S Vig, William A Alaynick, Joshua P Thaler, Chih-Cheng Tsai. EMBO J 2005
Akifumi Mizutani, Lei Wang, Harini Rajan, Parminder J S Vig, William A Alaynick, Joshua P Thaler, Chih-Cheng Tsai. EMBO J 2005
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SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein.
K Nakamura, S Y Jeong, T Uchihara, M Anno, K Nagashima, T Nagashima, S Ikeda, S Tsuji, I Kanazawa. Hum Mol Genet 2001
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Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy.
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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.
M DiFiglia, E Sapp, K O Chase, S W Davies, G P Bates, J P Vonsattel, N Aronin. Science 1997
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Cloning and developmental expression analysis of the murine homolog of the spinocerebellar ataxia type 1 gene (Sca1).
S Banfi, A Servadio, M Chung, F Capozzoli, L A Duvick, R Elde, H Y Zoghbi, H T Orr. Hum Mol Genet 1996
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Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.
Aaron B Bowman, Yung C Lam, Paymaan Jafar-Nejad, Hung-Kai Chen, Ronald Richman, Rodney C Samaco, John D Fryer, Juliette J Kahle, Harry T Orr, Huda Y Zoghbi. Nat Genet 2007
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Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2.
S M Pulst, A Nechiporuk, T Nechiporuk, S Gispert, X N Chen, I Lopes-Cendes, S Pearlman, S Starkman, G Orozco-Diaz, A Lunkes,[...]. Nat Genet 1996
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Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human.
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Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration.
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Phosphorylation of ATXN1 at Ser776 in the cerebellum.
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Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice.
C J Cummings, Y Sun, P Opal, B Antalffy, R Mestril, H T Orr, W H Dillmann, H Y Zoghbi. Hum Mol Genet 2001
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Ataxin-7 is a subunit of GCN5 histone acetyltransferase-containing complexes.
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Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion.
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Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration.
Vikas B Palhan, Shiming Chen, Guang-Hua Peng, Agneta Tjernberg, Armin M Gamper, Yuxin Fan, Brian T Chait, Albert R La Spada, Robert G Roeder. Proc Natl Acad Sci U S A 2005
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Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis.
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RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1.
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Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death.
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Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy.
Masahisa Katsuno, Hiroaki Adachi, Akito Kume, Mei Li, Yuji Nakagomi, Hisayoshi Niwa, Chen Sang, Yasushi Kobayashi, Manabu Doyu, Gen Sobue. Neuron 2002
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Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death.
Montserrat Arrasate, Siddhartha Mitra, Erik S Schweitzer, Mark R Segal, Steven Finkbeiner. Nature 2004
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The AXH module: an independently folded domain common to ataxin-1 and HBP1.
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Co-cited is the co-citation frequency, indicating how many articles cite the article together with the query article. Similarity is the co-citation as percentage of the times cited of the query article or the article in the search results, whichever is the lowest. These numbers are calculated for the last 100 citations when articles are cited more than 100 times.