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Times Cited: 620
Times Cited
Times Co-cited
Similarity
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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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.
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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.
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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
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Neuropathological classification of Huntington's disease.
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Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.
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Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.
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Huntington's disease: from molecular pathogenesis to clinical treatment.
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Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175.
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Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.
Christian Landles, Kirupa Sathasivam, Andreas Weiss, Ben Woodman, Hilary Moffitt, Steve Finkbeiner, Banghua Sun, Juliette Gafni, Lisa M Ellerby, Yvon Trottier,[...]. J Biol Chem 2010
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Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.
Michelle Gray, Dyna I Shirasaki, Carlos Cepeda, Véronique M André, Brian Wilburn, Xiao-Hong Lu, Jifang Tao, Irene Yamazaki, Shi-Hua Li, Yi E Sun,[...]. J Neurosci 2008
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Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.
Kirupa Sathasivam, Andreas Neueder, Theresa A Gipson, Christian Landles, Agnesska C Benjamin, Marie K Bondulich, Donna L Smith, Richard L M Faull, Raymund A C Roos, David Howland,[...]. Proc Natl Acad Sci U S A 2013
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Molecular mechanisms and potential therapeutical targets in Huntington's disease.
Chiara Zuccato, Marta Valenza, Elena Cattaneo. Physiol Rev 2010
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Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules.
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Huntington disease: natural history, biomarkers and prospects for therapeutics.
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Regional and cellular gene expression changes in human Huntington's disease brain.
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Huntington disease.
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Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice.
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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.
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The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation.
Yvette C Wong, Erika L F Holzbaur. J Neurosci 2014
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Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats.
Liliana B Menalled, Jessica D Sison, Ioannis Dragatsis, Scott Zeitlin, Marie-Françoise Chesselet. J Comp Neurol 2003
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Neurological abnormalities in a knock-in mouse model of Huntington's disease.
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Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease.
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Huntington aggregates may not predict neuronal death in Huntington's disease.
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Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients.
Edward J Wild, Roberto Boggio, Douglas Langbehn, Nicola Robertson, Salman Haider, James R C Miller, Henrik Zetterberg, Blair R Leavitt, Rainer Kuhn, Sarah J Tabrizi,[...]. J Clin Invest 2015
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Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse.
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The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease.
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Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease.
Brinda Ravikumar, Coralie Vacher, Zdenek Berger, Janet E Davies, Shouqing Luo, Lourdes G Oroz, Francesco Scaravilli, Douglas F Easton, Rainer Duden, Cahir J O'Kane,[...]. Nat Genet 2004
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10
Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin.
Chuan-En Wang, Hui Zhou, John R McGuire, Vincenzo Cerullo, Brendan Lee, Shi-Hua Li, Xiao-Jiang Li. J Cell Biol 2008
Chuan-En Wang, Hui Zhou, John R McGuire, Vincenzo Cerullo, Brendan Lee, Shi-Hua Li, Xiao-Jiang Li. J Cell Biol 2008
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Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo.
Justin Legleiter, Emily Mitchell, Gregor P Lotz, Ellen Sapp, Cheping Ng, Marian DiFiglia, Leslie M Thompson, Paul J Muchowski. J Biol Chem 2010
Justin Legleiter, Emily Mitchell, Gregor P Lotz, Ellen Sapp, Cheping Ng, Marian DiFiglia, Leslie M Thompson, Paul J Muchowski. J Biol Chem 2010
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Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.
K B Kegel, M Kim, E Sapp, C McIntyre, J G Castaño, N Aronin, M DiFiglia. J Neurosci 2000
K B Kegel, M Kim, E Sapp, C McIntyre, J G Castaño, N Aronin, M DiFiglia. J Neurosci 2000
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SUMO modification of Huntingtin and Huntington's disease pathology.
Joan S Steffan, Namita Agrawal, Judit Pallos, Erica Rockabrand, Lloyd C Trotman, Natalia Slepko, Katalin Illes, Tamas Lukacsovich, Ya-Zhen Zhu, Elena Cattaneo,[...]. Science 2004
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10
Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology.
E Scherzinger, A Sittler, K Schweiger, V Heiser, R Lurz, R Hasenbank, G P Bates, H Lehrach, E E Wanker. Proc Natl Acad Sci U S A 1999
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Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.
Y J Kim, Y Yi, E Sapp, Y Wang, B Cuiffo, K B Kegel, Z H Qin, N Aronin, M DiFiglia. Proc Natl Acad Sci U S A 2001
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Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila.
Shermali Gunawardena, Lu-Shiun Her, Richard G Brusch, Robert A Laymon, Ingrid R Niesman, Beth Gordesky-Gold, Louis Sintasath, Nancy M Bonini, Lawrence S B Goldstein. Neuron 2003
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10
Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions.
Astrid Lunkes, Katrin S Lindenberg, Léa Ben-Haïem, Chantal Weber, Didier Devys, G Bernhard Landwehrmeyer, Jean-Louis Mandel, Yvon Trottier. Mol Cell 2002
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Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis.
Holly B Kordasiewicz, Lisa M Stanek, Edward V Wancewicz, Curt Mazur, Melissa M McAlonis, Kimberly A Pytel, Jonathan W Artates, Andreas Weiss, Seng H Cheng, Lamya S Shihabuddin,[...]. Neuron 2012
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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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The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription.
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10
Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length.
M W Becher, J A Kotzuk, A H Sharp, S W Davies, G P Bates, D L Price, C A Ross. Neurobiol Dis 1998
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10
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release.
Yeun Su Choo, Gail V W Johnson, Marcy MacDonald, Peter J Detloff, Mathieu Lesort. Hum Mol Genet 2004
Yeun Su Choo, Gail V W Johnson, Marcy MacDonald, Peter J Detloff, Mathieu Lesort. Hum Mol Genet 2004
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N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.
Adam L Orr, Shihua Li, Chuan-En Wang, He Li, Jianjun Wang, Juan Rong, Xingshun Xu, Pier Giorgio Mastroberardino, J Timothy Greenamyre, Xiao-Jiang Li. J Neurosci 2008
Adam L Orr, Shihua Li, Chuan-En Wang, He Li, Jianjun Wang, Juan Rong, Xingshun Xu, Pier Giorgio Mastroberardino, J Timothy Greenamyre, Xiao-Jiang Li. J Neurosci 2008
9
Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease.
P Hemachandra Reddy, Ulziibat P Shirendeb. Biochim Biophys Acta 2012
P Hemachandra Reddy, Ulziibat P Shirendeb. Biochim Biophys Acta 2012
9
Potential function for the Huntingtin protein as a scaffold for selective autophagy.
Joseph Ochaba, Tamás Lukacsovich, George Csikos, Shuqiu Zheng, Julia Margulis, Lisa Salazar, Kai Mao, Alice L Lau, Sylvia Y Yeung, Sandrine Humbert,[...]. Proc Natl Acad Sci U S A 2014
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9
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro.
Eugenia Trushina, Roy B Dyer, John D Badger, Daren Ure, Lars Eide, David D Tran, Brent T Vrieze, Valerie Legendre-Guillemin, Peter S McPherson, Bhaskar S Mandavilli,[...]. Mol Cell Biol 2004
Eugenia Trushina, Roy B Dyer, John D Badger, Daren Ure, Lars Eide, David D Tran, Brent T Vrieze, Valerie Legendre-Guillemin, Peter S McPherson, Bhaskar S Mandavilli,[...]. Mol Cell Biol 2004
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.