Results 31 to 40 of about 44,176 (282)

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
core   +2 more sources

Huntingtin associates with the actin cytoskeleton and α-actinin isoforms to influence stimulus dependent morphology changes.

PLoS ONE, 2019
One response of cells to growth factor stimulus involves changes in morphology driven by the actin cytoskeleton and actin associated proteins which regulate functions such as cell adhesion, motility and in neurons, synaptic plasticity.
Adelaide Tousley, Maria Iuliano, Elizabeth Weisman, Ellen Sapp, Heather Richardson, Petr Vodicka, Jonathan Alexander, Neil Aronin, Marian DiFiglia, Kimberly B Kegel-Gleason   +9 more
doaj   +1 more source

Biomarkers for Huntington's disease: an update [PDF]

, 2012
Huntington's disease (HD) is a devastating autosomal-dominant neurodegenerative condition caused by a CAG repeat expansion in the gene encoding huntingtin which is characterised by progressive motor impairment, cognitive decline and neuropsychiatric ...
Scahill, RI, Tabrizi, SJ, Wild, EJ
core   +1 more source

NUB1 snubs huntingtin toxicity [PDF]

Nature Neuroscience, 2013
A screen now identifies a protein that regulates degradation of mutant huntingtin, which causes Huntington's disease, and manipulations show that promoting clearance of the toxic protein itself may be sufficient to halt disease.
Aron, Rebecca, Tsvetkov, Andrey, Finkbeiner, Steven   +2 more
openaire   +4 more sources

Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD.

PLoS ONE, 2015
Huntington's disease is a neurodegenerative disorder characterised primarily by motor abnormalities, and is caused by an expanded polyglutamine repeat in the huntingtin protein.
Kathryn R Bowles, Simon P Brooks, Stephen B Dunnett, Lesley Jones   +3 more
doaj   +1 more source

The HD Mutation Does Not Alter Neuronal Death in the Striatum of HdhQ92 Knock-in Mice after Mild Focal Ischemia

Neurobiology of Disease, 2002
Huntington's disease, with its dominant loss of striatal neurons, is triggered by an expanded glutamine tract in huntingtin. To investigate a proposed role for increased activation of the apoptotic cascade in mutant huntingtin's trigger mechanism, we ...
Shobu Namura, Lorenz Hirt, Vanessa C. Wheeler, Kim M. McGinnis, Paige Hilditch-Maguire, Michael A. Moskowitz, Marcy E. MacDonald, Francesca Persichetti   +7 more
doaj   +1 more source

Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice. [PDF]

, 2016
To gain insight into how mutant huntingtin (mHtt) CAG repeat length modifies Huntington's disease (HD) pathogenesis, we profiled mRNA in over 600 brain and peripheral tissue samples from HD knock-in mice with increasing CAG repeat lengths.
A Dobin, A Hodges, A Kuhn, A Pal, A Reiner, A Valencia, Andreas Tebbe, B Zhang, B Zhang, BM Bolstad, C Zuccato, CA Ross, Christoph Schaab, Daniel J Lavery, David Howland, DI Shirasaki, Doxa Chatzopoulou, DV Zaykin, EH Aylward, Eliana Marisa Ramos, ES Deneris, ES Lein, Fuying Gao, G Fishell, GA Smith, Giovanni Coppola, HT Orr, I Al-Ramahi, IS Seong, Ismael Al-Ramahi, J Cox, J Cox, J Labbadia, JA Miller, JC Jacobsen, JE Phillips, Jeffrey P Cantle, Jeffrey S Aaronson, JF Gusella, JF Gusella, Jim Rosinski, JM Dowen, JM Van Raamsdonk, JP Vonsattel, Juan Botas, K Becanovic, K Monahan, K Sharma, Karla El-Zein, LB Menalled, LB Menalled, LB Menalled, LS Kaltenbach, M Biagioli, M Heiman, M MACDONALD, M Mann, M Mielcarek, MA Pouladi, MC Oldham, MI Love, MK Lobo, MT Lin, N Wang, Nan Wang, P Giles, P Grange, P Langfelder, P Langfelder, P Langfelder, P Langfelder, P Langfelder, Peter Langfelder, PF Durrenberger, RC Gentleman, S Anders, S Horvath, S Toyoda, Sandeep Deverasetty, Seung Kwak, Steve Horvath, T Geiger, T Lu, WE Johnson, WV Chen, X Gu, X Gu, X William Yang, XH Lu, Xiao-Hong Lu, Y Benjamini, Y Guo, Y Shen, Y Wang, Yining Zhao   +94 more
core   +1 more source

The Biology of Huntingtin [PDF]

Neuron, 2016
Huntingtin (HTT) is now a famous protein because an abnormal expansion of a glutamine stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative disorder Huntington's disease (HD). The gene encoding huntingtin, HTT, and its dominantly inherited mutation were identified more than 20 years ago.
Saudou, Frédéric, Humbert, Sandrine
openaire   +2 more sources

Novel DNA Aptamers that Bind to Mutant Huntingtin and Modify Its Activity

Molecular Therapy: Nucleic Acids, 2018
The CAG repeat expansion that elongates the polyglutamine tract in huntingtin is the root genetic cause of Huntington’s disease (HD), a debilitating neurodegenerative disorder.
Baehyun Shin, Roy Jung, Hyejin Oh, Gwen E. Owens, Hyeongseok Lee, Seung Kwak, Ramee Lee, Susan L. Cotman, Jong-Min Lee, Marcy E. MacDonald, Ji-Joon Song, Ravi Vijayvargia, Ihn Sik Seong   +12 more
doaj   +1 more source

The Huntington disease protein accelerates breast tumour development and metastasis through ErbB2/HER2 signalling

EMBO Molecular Medicine, 2013
In Huntington disease (HD), polyglutamine expansion in the huntingtin protein causes specific neuronal death. The consequences of the presence of mutant huntingtin in other tissues are less well understood.
Cristovão Moreira Sousa, John Russel McGuire, Morgane Sonia Thion, David Gentien, Pierre de la Grange, Sophie Tezenas du Montcel, Anne Vincent‐Salomon, Alexandra Durr, Sandrine Humbert   +8 more
doaj   +1 more source