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
Times Cited: 146
Times Cited: 146
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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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
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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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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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
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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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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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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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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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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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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
18
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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18
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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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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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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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
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Generation and characterization of Sca2 (ataxin-2) knockout mice.
Tim-Rasmus Kiehl, Alex Nechiporuk, Karla P Figueroa, Mark T Keating, Duong P Huynh, Stefan-M Pulst. Biochem Biophys Res Commun 2006
Tim-Rasmus Kiehl, Alex Nechiporuk, Karla P Figueroa, Mark T Keating, Duong P Huynh, Stefan-M Pulst. Biochem Biophys Res Commun 2006
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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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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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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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SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.
Seung Yun Yoo, Mark E Pennesi, Edwin J Weeber, Bisong Xu, Richard Atkinson, Shiming Chen, Dawna L Armstrong, Samuel M Wu, J David Sweatt, Huda Y Zoghbi. Neuron 2003
Seung Yun Yoo, Mark E Pennesi, Edwin J Weeber, Bisong Xu, Richard Atkinson, Shiming Chen, Dawna L Armstrong, Samuel M Wu, J David Sweatt, Huda Y Zoghbi. Neuron 2003
14
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
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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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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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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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
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
13
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
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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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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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
12
Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles.
Jillian Friedrich, Holly B Kordasiewicz, Brennon O'Callaghan, Hillary P Handler, Carmen Wagener, Lisa Duvick, Eric E Swayze, Orion Rainwater, Bente Hofstra, Michael Benneyworth,[...]. JCI Insight 2018
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Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I.
M Y Chung, L P Ranum, L A Duvick, A Servadio, H Y Zoghbi, H T Orr. Nat Genet 1993
M Y Chung, L P Ranum, L A Duvick, A Servadio, H Y Zoghbi, H T Orr. Nat Genet 1993
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Loss of function of ATXN1 increases amyloid beta-protein levels by potentiating beta-secretase processing of beta-amyloid precursor protein.
Can Zhang, Andrew Browne, Daniel Child, Jason R Divito, Jesse A Stevenson, Rudolph E Tanzi. J Biol Chem 2010
Can Zhang, Andrew Browne, Daniel Child, Jason R Divito, Jesse A Stevenson, Rudolph E Tanzi. J Biol Chem 2010
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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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Deletions in chromosome 6p22.3-p24.3, including ATXN1, are associated with developmental delay and autism spectrum disorders.
Patrícia Bs Celestino-Soper, Cindy Skinner, Richard Schroer, Patricia Eng, Jayant Shenai, Malgorzata Mj Nowaczyk, Deborah Terespolsky, Donna Cushing, Gayle S Patel, Ladonna Immken,[...]. Mol Cytogenet 2012
Patrícia Bs Celestino-Soper, Cindy Skinner, Richard Schroer, Patricia Eng, Jayant Shenai, Malgorzata Mj Nowaczyk, Deborah Terespolsky, Donna Cushing, Gayle S Patel, Ladonna Immken,[...]. Mol Cytogenet 2012
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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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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
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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
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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease.
A Yamamoto, J J Lucas, R Hen. Cell 2000
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RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model.
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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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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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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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Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels.
Kei Watase, Curtis F Barrett, Taisuke Miyazaki, Taro Ishiguro, Kinya Ishikawa, Yuanxin Hu, Toshinori Unno, Yaling Sun, Sayumi Kasai, Masahiko Watanabe,[...]. Proc Natl Acad Sci U S A 2008
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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
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Brain pathology of spinocerebellar ataxias.
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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
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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.