Nathan D Jorgensen, J Michael Andresen, Sara Lagalwar, Ben Armstrong, Sam Stevens, Courtney E Byam, Lisa A Duvick, Shaojuan Lai, Paymaan Jafar-Nejad, Huda Y Zoghbi, H Brent Clark, Harry T Orr. J Neurochem 2009
Times Cited: 40
Times Cited: 40
Times Cited
Times Co-cited
Similarity
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
65
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
60
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
60
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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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
42
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
40
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
40
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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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
37
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
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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
35
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
32
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
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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
30
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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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
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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
27
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
27
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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Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy.
A R La Spada, E M Wilson, D B Lubahn, A E Harding, K H Fischbeck. Nature 1991
A R La Spada, E M Wilson, D B Lubahn, A E Harding, K H Fischbeck. Nature 1991
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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
25
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
22
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
22
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
22
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
22
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
22
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
Masahisa Katsuno, Hiroaki Adachi, Akito Kume, Mei Li, Yuji Nakagomi, Hisayoshi Niwa, Chen Sang, Yasushi Kobayashi, Manabu Doyu, Gen Sobue. Neuron 2002
20
SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal.
Brigit E Riley, Huda Y Zoghbi, Harry T Orr. J Biol Chem 2005
Brigit E Riley, Huda Y Zoghbi, Harry T Orr. J Biol Chem 2005
20
The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.
Sandrine Humbert, Elzbieta A Bryson, Fabrice P Cordelières, Nathan C Connors, Sandeep R Datta, Steven Finkbeiner, Michael E Greenberg, Frédéric Saudou. Dev Cell 2002
Sandrine Humbert, Elzbieta A Bryson, Fabrice P Cordelières, Nathan C Connors, Sandeep R Datta, Steven Finkbeiner, Michael E Greenberg, Frédéric Saudou. Dev Cell 2002
20
CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation.
Ismael Al-Ramahi, Yung C Lam, Hung-Kai Chen, Beatrice de Gouyon, Minghang Zhang, Alma M Pérez, Joana Branco, Maria de Haro, Cam Patterson, Huda Y Zoghbi,[...]. J Biol Chem 2006
Ismael Al-Ramahi, Yung C Lam, Hung-Kai Chen, Beatrice de Gouyon, Minghang Zhang, Alma M Pérez, Joana Branco, Maria de Haro, Cam Patterson, Huda Y Zoghbi,[...]. J Biol Chem 2006
20
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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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
20
A tissue-specific atlas of mouse protein phosphorylation and expression.
Edward L Huttlin, Mark P Jedrychowski, Joshua E Elias, Tapasree Goswami, Ramin Rad, Sean A Beausoleil, Judit Villén, Wilhelm Haas, Mathew E Sowa, Steven P Gygi. Cell 2010
Edward L Huttlin, Mark P Jedrychowski, Joshua E Elias, Tapasree Goswami, Ramin Rad, Sean A Beausoleil, Judit Villén, Wilhelm Haas, Mathew E Sowa, Steven P Gygi. Cell 2010
20
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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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
Montserrat Arrasate, Siddhartha Mitra, Erik S Schweitzer, Mark R Segal, Steven Finkbeiner. Nature 2004
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Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis.
Ludger Schöls, Peter Bauer, Thorsten Schmidt, Thorsten Schulte, Olaf Riess. Lancet Neurol 2004
Ludger Schöls, Peter Bauer, Thorsten Schmidt, Thorsten Schulte, Olaf Riess. Lancet Neurol 2004
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Bergmann glial S100B activates myo-inositol monophosphatase 1 and Co-localizes to purkinje cell vacuoles in SCA1 transgenic mice.
Parminder J S Vig, Qingmei Shao, S H Subramony, Mariper E Lopez, Eshan Safaya. Cerebellum 2009
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Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus.
D Lorenzetti, K Watase, B Xu, M M Matzuk, H T Orr, H Y Zoghbi. Hum Mol Genet 2000
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Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.
John M Warrick, Lance M Morabito, Julide Bilen, Beth Gordesky-Gold, Lynn Z Faust, Henry L Paulson, Nancy M Bonini. Mol Cell 2005
John M Warrick, Lance M Morabito, Julide Bilen, Beth Gordesky-Gold, Lynn Z Faust, Henry L Paulson, Nancy M Bonini. Mol Cell 2005
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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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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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Phosphorylation of ataxin-3 by glycogen synthase kinase 3beta at serine 256 regulates the aggregation of ataxin-3.
Erkang Fei, Nali Jia, Tao Zhang, Xiaochuan Ma, Hongfeng Wang, Chao Liu, Wei Zhang, Lili Ding, Nobuyuki Nukina, Guanghui Wang. Biochem Biophys Res Commun 2007
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CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3.
Thorsten Mueller, Peter Breuer, Ina Schmitt, Jochen Walter, Bernd O Evert, Ullrich Wüllner. Hum Mol Genet 2009
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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.
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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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CREB-binding protein sequestration by expanded polyglutamine.
A McCampbell, J P Taylor, A A Taye, J Robitschek, M Li, J Walcott, D Merry, Y Chai, H Paulson, G Sobue,[...]. Hum Mol Genet 2000
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Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes.
Nihar Ranjan Jana, Priyanka Dikshit, Anand Goswami, Svetlana Kotliarova, Shigeo Murata, Keiji Tanaka, Nobuyuki Nukina. J Biol Chem 2005
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Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.
Y Chai, S L Koppenhafer, S J Shoesmith, M K Perez, H L Paulson. Hum Mol Genet 1999
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15
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.