Huda Y Zoghbi, Harry T Orr. J Biol Chem 2009
Times Cited: 146
Times Cited: 146
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
29
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
28
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
27
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
24
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
23
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
22
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
19
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
17
Polyglutamine spinocerebellar ataxias - from genes to potential treatments.
Henry L Paulson, Vikram G Shakkottai, H Brent Clark, Harry T Orr. Nat Rev Neurosci 2017
Henry L Paulson, Vikram G Shakkottai, H Brent Clark, Harry T Orr. Nat Rev Neurosci 2017
17
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
16
Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis.
Juan Crespo-Barreto, John D Fryer, Chad A Shaw, Harry T Orr, Huda Y Zoghbi. PLoS Genet 2010
Juan Crespo-Barreto, John D Fryer, Chad A Shaw, Harry T Orr, Huda Y Zoghbi. PLoS Genet 2010
18
RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1.
Jeehye Park, Ismael Al-Ramahi, Qiumin Tan, Nissa Mollema, Javier R Diaz-Garcia, Tatiana Gallego-Flores, Hsiang-Chih Lu, Sarita Lagalwar, Lisa Duvick, Hyojin Kang,[...]. Nature 2013
Jeehye Park, Ismael Al-Ramahi, Qiumin Tan, Nissa Mollema, Javier R Diaz-Garcia, Tatiana Gallego-Flores, Hsiang-Chih Lu, Sarita Lagalwar, Lisa Duvick, Hyojin Kang,[...]. Nature 2013
17
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
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
15
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
14
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
14
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
13
Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals.
A Servadio, B Koshy, D Armstrong, B Antalffy, H T Orr, H Y Zoghbi. Nat Genet 1995
A Servadio, B Koshy, D Armstrong, B Antalffy, H T Orr, H Y Zoghbi. Nat Genet 1995
13
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
12
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
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
11
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
11
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
11
Polyglutamine is not all: the functional role of the AXH domain in the ataxin-1 protein.
Cesira de Chiara, Rajesh P Menon, Fabrizio Dal Piaz, Lesley Calder, Annalisa Pastore. J Mol Biol 2005
Cesira de Chiara, Rajesh P Menon, Fabrizio Dal Piaz, Lesley Calder, Annalisa Pastore. J Mol Biol 2005
17
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
11
Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7.
Udo Rüb, Ludger Schöls, Henry Paulson, Georg Auburger, Pawel Kermer, Joanna C Jen, Kay Seidel, Horst-Werner Korf, Thomas Deller. Prog Neurobiol 2013
Udo Rüb, Ludger Schöls, Henry Paulson, Georg Auburger, Pawel Kermer, Joanna C Jen, Kay Seidel, Horst-Werner Korf, Thomas Deller. Prog Neurobiol 2013
11
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
10
The structure of the AXH domain of spinocerebellar ataxin-1.
Yu Wai Chen, Mark D Allen, Dmitry B Veprintsev, Jan Löwe, Mark Bycroft. J Biol Chem 2004
Yu Wai Chen, Mark D Allen, Dmitry B Veprintsev, Jan Löwe, Mark Bycroft. J Biol Chem 2004
21
Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.
Melissa Ingram, Emily A L Wozniak, Lisa Duvick, Rendong Yang, Paul Bergmann, Robert Carson, Brennon O'Callaghan, Huda Y Zoghbi, Christine Henzler, Harry T Orr. Neuron 2016
Melissa Ingram, Emily A L Wozniak, Lisa Duvick, Rendong Yang, Paul Bergmann, Robert Carson, Brennon O'Callaghan, Huda Y Zoghbi, Christine Henzler, Harry T Orr. Neuron 2016
17
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
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
9
9
Clinical, genetic, molecular, and pathophysiological insights into spinocerebellar ataxia type 1.
Antoni Matilla-Dueñas, Robert Goold, Paola Giunti. Cerebellum 2008
Antoni Matilla-Dueñas, Robert Goold, Paola Giunti. Cerebellum 2008
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Brain pathology of spinocerebellar ataxias.
Kay Seidel, Sonny Siswanto, Ewout R P Brunt, Wilfred den Dunnen, Horst-Werner Korf, Udo Rüb. Acta Neuropathol 2012
Kay Seidel, Sonny Siswanto, Ewout R P Brunt, Wilfred den Dunnen, Horst-Werner Korf, Udo Rüb. Acta Neuropathol 2012
9
The AXH module: an independently folded domain common to ataxin-1 and HBP1.
C de Chiara, C Giannini, S Adinolfi, J de Boer, S Guida, A Ramos, C Jodice, D Kioussis, A Pastore. FEBS Lett 2003
C de Chiara, C Giannini, S Adinolfi, J de Boer, S Guida, A Ramos, C Jodice, D Kioussis, A Pastore. FEBS Lett 2003
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Repeat expansion disease: progress and puzzles in disease pathogenesis.
Albert R La Spada, J Paul Taylor. Nat Rev Genet 2010
Albert R La Spada, J Paul Taylor. Nat Rev Genet 2010
9
Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration.
Paymaan Jafar-Nejad, Christopher S Ward, Ronald Richman, Harry T Orr, Huda Y Zoghbi. Proc Natl Acad Sci U S A 2011
Paymaan Jafar-Nejad, Christopher S Ward, Ronald Richman, Harry T Orr, Huda Y Zoghbi. Proc Natl Acad Sci U S A 2011
17
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
9
Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua.
Eunji Kim, Hsiang-Chih Lu, Huda Y Zoghbi, Ji-Joon Song. Genes Dev 2013
Eunji Kim, Hsiang-Chih Lu, Huda Y Zoghbi, Ji-Joon Song. Genes Dev 2013
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14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus.
Shaojuan Lai, Brennon O'Callaghan, Huda Y Zoghbi, Harry T Orr. J Biol Chem 2011
Shaojuan Lai, Brennon O'Callaghan, Huda Y Zoghbi, Harry T Orr. J Biol Chem 2011
22
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
9
CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1.
Y Kawaguchi, T Okamoto, M Taniwaki, M Aizawa, M Inoue, S Katayama, H Kawakami, S Nakamura, M Nishimura, I Akiguchi. Nat Genet 1994
Y Kawaguchi, T Okamoto, M Taniwaki, M Aizawa, M Inoue, S Katayama, H Kawakami, S Nakamura, M Nishimura, I Akiguchi. Nat Genet 1994
9
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.
Andrew C Elden, Hyung-Jun Kim, Michael P Hart, Alice S Chen-Plotkin, Brian S Johnson, Xiaodong Fang, Maria Armakola, Felix Geser, Robert Greene, Min Min Lu,[...]. Nature 2010
Andrew C Elden, Hyung-Jun Kim, Michael P Hart, Alice S Chen-Plotkin, Brian S Johnson, Xiaodong Fang, Maria Armakola, Felix Geser, Robert Greene, Min Min Lu,[...]. Nature 2010
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Broad therapeutic benefit after RNAi expression vector delivery to deep cerebellar nuclei: implications for spinocerebellar ataxia type 1 therapy.
Megan S Keiser, Ryan L Boudreau, Beverly L Davidson. Mol Ther 2014
Megan S Keiser, Ryan L Boudreau, Beverly L Davidson. Mol Ther 2014
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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.
Maxime W C Rousseaux, Tyler Tschumperlin, Hsiang-Chih Lu, Elizabeth P Lackey, Vitaliy V Bondar, Ying-Wooi Wan, Qiumin Tan, Carolyn J Adamski, Jillian Friedrich, Kirk Twaroski,[...]. Neuron 2018
Maxime W C Rousseaux, Tyler Tschumperlin, Hsiang-Chih Lu, Elizabeth P Lackey, Vitaliy V Bondar, Ying-Wooi Wan, Qiumin Tan, Carolyn J Adamski, Jillian Friedrich, Kirk Twaroski,[...]. Neuron 2018
20
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
8
SCA1-phosphorylation, a regulator of Ataxin-1 function and pathogenesis.
Harry T Orr. Prog Neurobiol 2012
Harry T Orr. Prog Neurobiol 2012
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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
16
A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration.
Janghoo Lim, Tong Hao, Chad Shaw, Akash J Patel, Gábor Szabó, Jean-François Rual, C Joseph Fisk, Ning Li, Alex Smolyar, David E Hill,[...]. Cell 2006
Janghoo Lim, Tong Hao, Chad Shaw, Akash J Patel, Gábor Szabó, Jean-François Rual, C Joseph Fisk, Ning Li, Alex Smolyar, David E Hill,[...]. Cell 2006
8
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.