Céline Ruegsegger, David M Stucki, Silvio Steiner, Nico Angliker, Julika Radecke, Eva Keller, Benoît Zuber, Markus A Rüegg, Smita Saxena. Neuron 2016
Times Cited: 22
Times Cited: 22
Times Cited
Times Co-cited
Similarity
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
40
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
40
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
40
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
36
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
36
Abnormalities in the climbing fiber-Purkinje cell circuitry contribute to neuronal dysfunction in ATXN1[82Q] mice.
Justin A Barnes, Blake A Ebner, Lisa A Duvick, Wangcai Gao, Gang Chen, Harry T Orr, Timothy J Ebner. J Neurosci 2011
Justin A Barnes, Blake A Ebner, Lisa A Duvick, Wangcai Gao, Gang Chen, Harry T Orr, Timothy J Ebner. J Neurosci 2011
36
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
36
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
31
Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum.
Blake A Ebner, Melissa A Ingram, Justin A Barnes, Lisa A Duvick, Jill L Frisch, H Brent Clark, Huda Y Zoghbi, Timothy J Ebner, Harry T Orr. J Neurosci 2013
Blake A Ebner, Melissa A Ingram, Justin A Barnes, Lisa A Duvick, Jill L Frisch, H Brent Clark, Huda Y Zoghbi, Timothy J Ebner, Harry T Orr. J Neurosci 2013
31
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
27
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
22
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
22
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
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
22
Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability.
James M Dell'Orco, Aaron H Wasserman, Ravi Chopra, Melissa A C Ingram, Yuan-Shih Hu, Vikrant Singh, Heike Wulff, Puneet Opal, Harry T Orr, Vikram G Shakkottai. J Neurosci 2015
James M Dell'Orco, Aaron H Wasserman, Ravi Chopra, Melissa A C Ingram, Yuan-Shih Hu, Vikrant Singh, Heike Wulff, Puneet Opal, Harry T Orr, Vikram G Shakkottai. J Neurosci 2015
22
Aminopyridines correct early dysfunction and delay neurodegeneration in a mouse model of spinocerebellar ataxia type 1.
Raphael Hourez, Laurent Servais, David Orduz, David Gall, Isabelle Millard, Alban de Kerchove d'Exaerde, Guy Cheron, Harry T Orr, Massimo Pandolfo, Serge N Schiffmann. J Neurosci 2011
Raphael Hourez, Laurent Servais, David Orduz, David Gall, Isabelle Millard, Alban de Kerchove d'Exaerde, Guy Cheron, Harry T Orr, Massimo Pandolfo, Serge N Schiffmann. J Neurosci 2011
22
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
18
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
18
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
18
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
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
18
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
18
Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.
A Matilla-Dueñas, T Ashizawa, A Brice, S Magri, K N McFarland, M Pandolfo, S M Pulst, O Riess, D C Rubinsztein, J Schmidt,[...]. Cerebellum 2014
A Matilla-Dueñas, T Ashizawa, A Brice, S Magri, K N McFarland, M Pandolfo, S M Pulst, O Riess, D C Rubinsztein, J Schmidt,[...]. Cerebellum 2014
18
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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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
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Trimmomatic: a flexible trimmer for Illumina sequence data.
Anthony M Bolger, Marc Lohse, Bjoern Usadel. Bioinformatics 2014
Anthony M Bolger, Marc Lohse, Bjoern Usadel. Bioinformatics 2014
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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
13
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
13
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
13
Phosphorylation of ATXN1 at Ser776 in the cerebellum.
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,[...]. J Neurochem 2009
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,[...]. J Neurochem 2009
13
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
13
Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model.
Kristin M Gehrking, J Michael Andresen, Lisa Duvick, John Lough, Huda Y Zoghbi, Harry T Orr. Hum Mol Genet 2011
Kristin M Gehrking, J Michael Andresen, Lisa Duvick, John Lough, Huda Y Zoghbi, Harry T Orr. Hum Mol Genet 2011
13
Climbing fibers in spinocerebellar ataxia: A mechanism for the loss of motor control.
C J L M Smeets, D S Verbeek. Neurobiol Dis 2016
C J L M Smeets, D S Verbeek. Neurobiol Dis 2016
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HMGB1 facilitates repair of mitochondrial DNA damage and extends the lifespan of mutant ataxin-1 knock-in mice.
Hikaru Ito, Kyota Fujita, Kazuhiko Tagawa, Xigui Chen, Hidenori Homma, Toshikazu Sasabe, Jun Shimizu, Shigeomi Shimizu, Takuya Tamura, Shin-ichi Muramatsu,[...]. EMBO Mol Med 2015
Hikaru Ito, Kyota Fujita, Kazuhiko Tagawa, Xigui Chen, Hidenori Homma, Toshikazu Sasabe, Jun Shimizu, Shigeomi Shimizu, Takuya Tamura, Shin-ichi Muramatsu,[...]. EMBO Mol Med 2015
13
Chronic suppression of inositol 1,4,5-triphosphate receptor-mediated calcium signaling in cerebellar purkinje cells alleviates pathological phenotype in spinocerebellar ataxia 2 mice.
Adebimpe W Kasumu, Xia Liang, Polina Egorova, Daria Vorontsova, Ilya Bezprozvanny. J Neurosci 2012
Adebimpe W Kasumu, Xia Liang, Polina Egorova, Daria Vorontsova, Ilya Bezprozvanny. J Neurosci 2012
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mGluR1 in cerebellar Purkinje cells essential for long-term depression, synapse elimination, and motor coordination.
T Ichise, M Kano, K Hashimoto, D Yanagihara, K Nakao, R Shigemoto, M Katsuki, A Aiba. Science 2000
T Ichise, M Kano, K Hashimoto, D Yanagihara, K Nakao, R Shigemoto, M Katsuki, A Aiba. Science 2000
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Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond.
Alexandra Durr. Lancet Neurol 2010
Alexandra Durr. Lancet Neurol 2010
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Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2.
Stephen T Hansen, Pratap Meera, Thomas S Otis, Stefan M Pulst. Hum Mol Genet 2013
Stephen T Hansen, Pratap Meera, Thomas S Otis, Stefan M Pulst. Hum Mol Genet 2013
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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
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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
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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
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STAR: ultrafast universal RNA-seq aligner.
Alexander Dobin, Carrie A Davis, Felix Schlesinger, Jorg Drenkow, Chris Zaleski, Sonali Jha, Philippe Batut, Mark Chaisson, Thomas R Gingeras. Bioinformatics 2013
Alexander Dobin, Carrie A Davis, Felix Schlesinger, Jorg Drenkow, Chris Zaleski, Sonali Jha, Philippe Batut, Mark Chaisson, Thomas R Gingeras. Bioinformatics 2013
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Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1.
M Cvetanovic, M Ingram, H Orr, P Opal. Neuroscience 2015
M Cvetanovic, M Ingram, H Orr, P Opal. Neuroscience 2015
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Retrograde BDNF to TrkB signaling promotes synapse elimination in the developing cerebellum.
Myeongjeong Choo, Taisuke Miyazaki, Maya Yamazaki, Meiko Kawamura, Takanobu Nakazawa, Jianling Zhang, Asami Tanimura, Naofumi Uesaka, Masahiko Watanabe, Kenji Sakimura,[...]. Nat Commun 2017
Myeongjeong Choo, Taisuke Miyazaki, Maya Yamazaki, Meiko Kawamura, Takanobu Nakazawa, Jianling Zhang, Asami Tanimura, Naofumi Uesaka, Masahiko Watanabe, Kenji Sakimura,[...]. Nat Commun 2017
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Reduced brain-derived neurotrophic factor (BDNF) mRNA expression and presence of BDNF-immunoreactive granules in the spinocerebellar ataxia type 6 (SCA6) cerebellum.
Makoto Takahashi, Kinya Ishikawa, Nozomu Sato, Masato Obayashi, Yusuke Niimi, Taro Ishiguro, Mitsunori Yamada, Yasuko Toyoshima, Hitoshi Takahashi, Takeo Kato,[...]. Neuropathology 2012
Makoto Takahashi, Kinya Ishikawa, Nozomu Sato, Masato Obayashi, Yusuke Niimi, Taro Ishiguro, Mitsunori Yamada, Yasuko Toyoshima, Hitoshi Takahashi, Takeo Kato,[...]. Neuropathology 2012
23
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
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
13
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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Transcript assembly and quantification by RNA-Seq reveals unannotated transcripts and isoform switching during cell differentiation.
Cole Trapnell, Brian A Williams, Geo Pertea, Ali Mortazavi, Gordon Kwan, Marijke J van Baren, Steven L Salzberg, Barbara J Wold, Lior Pachter. Nat Biotechnol 2010
Cole Trapnell, Brian A Williams, Geo Pertea, Ali Mortazavi, Gordon Kwan, Marijke J van Baren, Steven L Salzberg, Barbara J Wold, Lior Pachter. Nat Biotechnol 2010
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Emerging pathogenic pathways in the spinocerebellar ataxias.
Kerri M Carlson, J Michael Andresen, Harry T Orr. Curr Opin Genet Dev 2009
Kerri M Carlson, J Michael Andresen, Harry T Orr. Curr Opin Genet Dev 2009
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TopHat2: accurate alignment of transcriptomes in the presence of insertions, deletions and gene fusions.
Daehwan Kim, Geo Pertea, Cole Trapnell, Harold Pimentel, Ryan Kelley, Steven L Salzberg. Genome Biol 2013
Daehwan Kim, Geo Pertea, Cole Trapnell, Harold Pimentel, Ryan Kelley, Steven L Salzberg. Genome Biol 2013
9
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.