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Times Cited: 1748
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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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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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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Neuropathological classification of Huntington's disease.
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In Situ Architecture and Cellular Interactions of PolyQ Inclusions.
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Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.
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Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.
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Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease.
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Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.
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Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
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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.
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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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Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.
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Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism.
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Potential function for the Huntingtin protein as a scaffold for selective autophagy.
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Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes.
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Targeting Huntingtin Expression in Patients with Huntington's Disease.
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Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington's disease.
Taneli Heikkinen, Kimmo Lehtimäki, Nina Vartiainen, Jukka Puoliväli, Susan J Hendricks, Jack R Glaser, Amyaouch Bradaia, Kristian Wadel, Chrystelle Touller, Outi Kontkanen,[...]. PLoS One 2012
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9
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice.
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Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization.
Michelle A Poirier, Huilin Li, Jed Macosko, Shuowei Cai, Mario Amzel, Christopher A Ross. J Biol Chem 2002
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Mutational analysis implicates the amyloid fibril as the toxic entity in Huntington's disease.
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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.
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Spatiotemporal Proteomic Profiling of Huntington's Disease Inclusions Reveals Widespread Loss of Protein Function.
Fabian Hosp, Sara Gutiérrez-Ángel, Martin H Schaefer, Jürgen Cox, Felix Meissner, Mark S Hipp, F-Ulrich Hartl, Rüdiger Klein, Irina Dudanova, Matthias Mann. Cell Rep 2017
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A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.
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Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation.
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Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release.
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Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool.
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mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease.
Anne Ast, Alexander Buntru, Franziska Schindler, Regine Hasenkopf, Aline Schulz, Lydia Brusendorf, Konrad Klockmeier, Gerlinde Grelle, Benjamin McMahon, Hannah Niederlechner,[...]. Mol Cell 2018
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8
Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease.
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Huntingtin Lowering Strategies for Disease Modification in Huntington's Disease.
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Neurological abnormalities in a knock-in mouse model of Huntington's disease.
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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease.
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Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice.
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Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity.
Randy Singh Atwal, Jianrun Xia, Deborah Pinchev, Jillian Taylor, Richard M Epand, Ray Truant. Hum Mol Genet 2007
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IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.
Leslie Michels Thompson, Charity T Aiken, Linda S Kaltenbach, Namita Agrawal, Katalin Illes, Ali Khoshnan, Marta Martinez-Vincente, Montserrat Arrasate, Jacqueline Gire O'Rourke, Hasan Khashwji,[...]. J Cell Biol 2009
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Critical nucleus size for disease-related polyglutamine aggregation is repeat-length dependent.
Karunakar Kar, Murali Jayaraman, Bankanidhi Sahoo, Ravindra Kodali, Ronald Wetzel. Nat Struct Mol Biol 2011
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Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice.
Peter Langfelder, Jeffrey P Cantle, Doxa Chatzopoulou, Nan Wang, Fuying Gao, Ismael Al-Ramahi, Xiao-Hong Lu, Eliana Marisa Ramos, Karla El-Zein, Yining Zhao,[...]. Nat Neurosci 2016
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7
Huntingtin functions as a scaffold for selective macroautophagy.
Yan-Ning Rui, Zhen Xu, Bindi Patel, Zhihua Chen, Dongsheng Chen, Antonio Tito, Gabriela David, Yamin Sun, Erin F Stimming, Hugo J Bellen,[...]. Nat Cell Biol 2015
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Allele-selective lowering of mutant HTT protein by HTT-LC3 linker compounds.
Zhaoyang Li, Cen Wang, Ziying Wang, Chenggang Zhu, Jie Li, Tian Sha, Lixiang Ma, Chao Gao, Yi Yang, Yimin Sun,[...]. Nature 2019
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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.