Juan Crespo-Barreto, John D Fryer, Chad A Shaw, Harry T Orr, Huda Y Zoghbi. PLoS Genet 2010
Times Cited: 86
Times Cited: 86
Times Cited
Times Co-cited
Similarity
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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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
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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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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
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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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
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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Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua.
John D Fryer, Peng Yu, Hyojin Kang, Caleigh Mandel-Brehm, Angela N Carter, Juan Crespo-Barreto, Yan Gao, Adriano Flora, Chad Shaw, Harry T Orr,[...]. Science 2011
John D Fryer, Peng Yu, Hyojin Kang, Caleigh Mandel-Brehm, Angela N Carter, Juan Crespo-Barreto, Yan Gao, Adriano Flora, Chad Shaw, Harry T Orr,[...]. Science 2011
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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
Huda Y Zoghbi, Harry T Orr. J Biol Chem 2009
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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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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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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
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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
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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
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ATXN1 protein family and CIC regulate extracellular matrix remodeling and lung alveolarization.
Yoontae Lee, John D Fryer, Hyojin Kang, Juan Crespo-Barreto, Aaron B Bowman, Yan Gao, Juliette J Kahle, Jeong Soo Hong, Farrah Kheradmand, Harry T Orr,[...]. Dev Cell 2011
Yoontae Lee, John D Fryer, Hyojin Kang, Juan Crespo-Barreto, Aaron B Bowman, Yan Gao, Juliette J Kahle, Jeong Soo Hong, Farrah Kheradmand, Harry T Orr,[...]. Dev Cell 2011
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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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Down-regulation of the dopamine receptor D2 in mice lacking ataxin 1.
Robert Goold, Michael Hubank, Abigail Hunt, Janice Holton, Rajesh P Menon, Tamas Revesz, Massimo Pandolfo, Antoni Matilla-Dueñas. Hum Mol Genet 2007
Robert Goold, Michael Hubank, Abigail Hunt, Janice Holton, Rajesh P Menon, Tamas Revesz, Massimo Pandolfo, Antoni Matilla-Dueñas. Hum Mol Genet 2007
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Fusion between CIC and DUX4 up-regulates PEA3 family genes in Ewing-like sarcomas with t(4;19)(q35;q13) translocation.
Miho Kawamura-Saito, Yukari Yamazaki, Keiko Kaneko, Noriyoshi Kawaguchi, Hiroaki Kanda, Hiroyuki Mukai, Takahiro Gotoh, Tohru Motoi, Masashi Fukayama, Hiroyuki Aburatani,[...]. Hum Mol Genet 2006
Miho Kawamura-Saito, Yukari Yamazaki, Keiko Kaneko, Noriyoshi Kawaguchi, Hiroaki Kanda, Hiroyuki Mukai, Takahiro Gotoh, Tohru Motoi, Masashi Fukayama, Hiroyuki Aburatani,[...]. Hum Mol Genet 2006
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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
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Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.
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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
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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Vascular endothelial growth factor ameliorates the ataxic phenotype in a mouse model of spinocerebellar ataxia type 1.
Marija Cvetanovic, Jay M Patel, Hugo H Marti, Ameet R Kini, Puneet Opal. Nat Med 2011
Marija Cvetanovic, Jay M Patel, Hugo H Marti, Ameet R Kini, Puneet Opal. Nat Med 2011
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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
17
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
16
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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Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2.
Jing Liu, Tie-Shan Tang, Huiping Tu, Omar Nelson, Emily Herndon, Duong P Huynh, Stefan M Pulst, Ilya Bezprozvanny. J Neurosci 2009
Jing Liu, Tie-Shan Tang, Huiping Tu, Omar Nelson, Emily Herndon, Duong P Huynh, Stefan M Pulst, Ilya Bezprozvanny. J Neurosci 2009
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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
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15
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
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A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration.
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Ataxin-1 and Brother of ataxin-1 are components of the Notch signalling pathway.
Xin Tong, Hongxing Gui, Feng Jin, Bryan W Heck, Peihui Lin, Jianjie Ma, Joseph D Fondell, Chih-Cheng Tsai. EMBO Rep 2011
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Phosphorylation of S776 and 14-3-3 binding modulate ataxin-1 interaction with splicing factors.
Cesira de Chiara, Rajesh P Menon, Molly Strom, Toby J Gibson, Annalisa Pastore. PLoS One 2009
Cesira de Chiara, Rajesh P Menon, Molly Strom, Toby J Gibson, Annalisa Pastore. PLoS One 2009
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Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation.
An-Hsun Chou, Tu-Hsueh Yeh, Pin Ouyang, Ying-Ling Chen, Si-Ying Chen, Hung-Li Wang. Neurobiol Dis 2008
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The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7.
Jennifer R Gatchel, Kei Watase, Christina Thaller, James P Carson, Paymaan Jafar-Nejad, Chad Shaw, Tao Zu, Harry T Orr, Huda Y Zoghbi. Proc Natl Acad Sci U S A 2008
Jennifer R Gatchel, Kei Watase, Christina Thaller, James P Carson, Paymaan Jafar-Nejad, Chad Shaw, Tao Zu, Harry T Orr, Huda Y Zoghbi. Proc Natl Acad Sci U S A 2008
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Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2.
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Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel.
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Relief of gene repression by torso RTK signaling: role of capicua in Drosophila terminal and dorsoventral patterning.
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ERK/p90(RSK)/14-3-3 signalling has an impact on expression of PEA3 Ets transcription factors via the transcriptional repressor capicúa.
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Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua.
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Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2.
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Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.
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ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism.
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Identification of genes that modify ataxin-1-induced neurodegeneration.
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CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1.
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Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model.
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Mutations in CIC and FUBP1 contribute to human oligodendroglioma.
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RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.
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Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.
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Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3.
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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.