Results 21 to 30 of about 33,531 (157)

The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins [PDF]

Cells, 2021
Protein misfolding and aggregation are implicated in many neurodegenerative diseases. One of these diseases is Huntington’s, which is caused by increased glutamine-encoding trinucleotide repeats within the Huntingtin gene. Like other misfolded proteins, mutated Huntingtin proteins with polyglutamine expansions are prone to aggregation.
Yanchang Wang, Austin Folger
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N17 Modifies mutant Huntingtin nuclear pathogenesis and severity of disease in HD BAC transgenic mice. [PDF]

, 2015
The nucleus is a critical subcellular compartment for the pathogenesis of polyglutamine disorders, including Huntington's disease (HD). Recent studies suggest the first 17-amino-acid domain (N17) of mutant huntingtin (mHTT) mediates its nuclear exclusion
Barth, Albert M, Cantle, Jeffrey P, Coppola, Giovanni, Diamond, Marc, Gao, Fuying, Greiner, Erin R, Gu, Xiaofeng, Lee, CY Daniel, Mody, Istvan, Park, Chang Sin, Sandoval-Miller, Susana, Yang, X William, Zhang, Richard L, Zhang, Zhiqiang   +13 more
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The Huntington's disease mutation impairs Huntingtin's role in the transport of NF-κB from the synapse to the nucleus [PDF]

, 2010
Expansion of a polyglutamine (polyQ) tract in the Huntingtin (Htt) protein causes Huntington's disease (HD), a fatal inherited neurodegenerative disorder.
Baquet, Bates, Bennett, Bhakar, Blahos, Caviston, Chen, Cho, Colin, Colomer, Dragatsis, Dunkley, Edoardo Marcora, Engelender, Fagerlund, Fridmacher, Gauthier, Graham, Gray, Gutekunst, Hanz, Harkiolaki, Hayden, Hering, Hickey, Hunt, Imarisio, Jordan, Joshi, Kaltschmidt, Kaltschmidt, Kaltschmidt, Kennedy, Korkin, Kornau, Leavitt, Leavitt, Li, Li, Li, Lipsky, Ma, Marcora, Marini, Marini, Mary B. Kennedy, Mattson, Meffert, Meffert, Menalled, Mikenberg, Mosammaparast, Otis, Patterson, Perlson, Shimojo, Simmons, Song, Strand, Sun, Suzuki, Takano, The Huntington's Disease Collaborative Research Group, Thompson, Tian, Towbin, Trushina, Velier, Wang, Wellmann, Xie, Zhang, Zuccato, Zuccato, Zuccato   +74 more
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Metabolic and transcriptomic analysis of Huntington's disease model reveal changes in intracellular glucose levels and related genes. [PDF]

, 2017
Huntington's Disease (HD) is a neurodegenerative disorder caused by an expansion in a CAG-tri-nucleotide repeat that introduces a poly-glutamine stretch into the huntingtin protein (mHTT).
Chaves, Gepoliano, Costa, Yvonne Da, Hadiprodjo, Hana, Pourmand, Nader, Rao, Namrata V, Tokuno, Zachary, Özel, Rıfat Emrah   +6 more
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Effects of overexpression of Huntingtin proteins on mitochondrial integrity [PDF]

Human Molecular Genetics, 2008
Huntington's disease (HD) is caused by an expansion of a CAG trinucleotide sequence that encodes a polyglutamine tract in the huntingtin (Htt) protein. Expansion of the polyglutamine tract above 35 repeats causes disease, with the age of onset inversely related to the degree of expansion above this number.
Hongmin Wang, Precious J. Lim, Mariusz Karbowski, Mervyn J. Monteiro   +3 more
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IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome [PDF]

, 2009
Expansion of the polyglutamine repeat within the protein Huntingtin (Htt) causes Huntington's disease, a neurodegenerative disease associated with aging and the accumulation of mutant Htt in diseased neurons.
Aiken, Ali Khoshnan, Alice L. Lau, Ana Maria Cuervo, Anne, Apostol, Arrasate, Arrasate, Ashish Massey, Atwal, Atwal, Björkqvist, Brown, Charity T. Aiken, Chen, Chondrogianni, Cornett, Cuervo, Cuervo, Cuervo, D'Orazi, Dan, Donald C. Lo, Fang, Finkbeiner, Frank M. LaFerla, Gillian Bates, Graham, Grossman, Gu, Guerrero, Gutekunst, Hasan Khashwji, Henn, Hernandez-Hernandez, Hietakangas, Hofmann, Hu, Huang, Humbert, Hunter, J. Lawrence Marsh, Jacqueline Gire O'Rourke, Jeong, Jiang, Joan S. Steffan, Karin, Karin, Katalin Illes, Kaushik, Kazantsev, Khoshnan, Khoshnan, Kim N. Green, Komatsu, Lan Huang, Leslie Michels Thompson, Linda S. Kaltenbach, Liu, Lo, Luo, Mangiarini, Marta Martinez-Vincente, Martinez-Vicente, Massey, Massey, Michael R. Hayden, Michels, Mizushima, Montserrat Arrasate, Namita Agrawal, Orr, Paul H. Patterson, Peters-Libeu, Rockabrand, Saudou, Schilling, Scott O. Zeitlin, Shao, Southwell, Steffan, Steffan, Steffan, Steven Finkbeiner, Tagwerker, Tamas Lukacsovich, Tonoki, Verma, Walker, Warby, Warby, Warby, Wu, Xia, Ya-Zhen Zhu, Yanai, Zhang, Zuccato   +97 more
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Inhibition of mitochondrial protein import by mutant huntingtin [PDF]

Nature Neuroscience, 2014
Mitochondrial dysfunction is associated with neuronal loss in Huntington's disease (HD), a neurodegenerative disease caused by an abnormal polyglutamine expansion in huntingtin (Htt). However, the mechanisms linking mutant Htt and mitochondrial dysfunction in HD remain unknown.
Sergei V. Baranov, Oxana V. Baranova, Robert J. Ferrante, Yanchun Pan, Robert M. Friedlander, Diane L. Carlisle, Jinho Kim, Albert H. Kim, Hiroko Yano, Svitlana Yablonska   +9 more
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Sphingomyelin and GM1 Influence Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes [PDF]

, 2018
Huntington disease (HD) is an inherited neurodegenerative disease caused by the expansion beyond a critical threshold of a polyglutamine (polyQ) tract near the N-terminus of the huntingtin (htt) protein. Expanded polyQ promotes the formation of a variety
Campbell, Warren A., Chaibva, Maxmore, Frey, Shelli L., Gao, Xiang, Jain, Pranav, Legleiter, Justin   +5 more
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Forms and Phases in Huntingtin Protein Aggregation [PDF]

Molecular Cell, 2018
Using a combination of fluorescence microscopy and electron tomography, Peskett et al. (2018), in this issue of Molecular Cell, explore the nucleation of amyloid-like filaments from liquid-like condensates of huntingtin protein exon1 with disease-related polyQ extensions.
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Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity [PDF]

, 2015
Assemblies of huntingtin (HTT) fragments with expanded polyglutamine (polyQ) tracts are a pathological hallmark of Huntington's disease (HD). The molecular mechanisms by which these structures are formed and cause neuronal dysfunction and toxicity are ...
Andrade-Navarro, Miguel A., Bates, Gillian P., Boeddrich, Annett, Bounab, Yacine, Chaurasia, Gautam, Fournier, David, Friedrich, Ralf P., Futschik, Matthias E., Graham, Rona K., Haenig, Christian, Hayden, Michael R., Hesse, Franziska, Klockmeier, Konrad, Li, Shuang, Nicoletti, Cecilia, Priller, Josef, Riechers, Sean-Patrick, Russ, Jenny, Schnoegl, Sigrid, Sigrist, Stephan, Strempel, Nadine, Stroedicke, Martin, Wanker, Erich E., Wiglenda, Thomas, Yigit, Sargon   +24 more
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