Results 51 to 60 of about 5,553,804 (280)

Modulation of Huntingtin Toxicity by BAG1 is Dependent on an Intact BAG Domain

Molecules, 2010
Huntington´s disease, one of the so-called poly-glutamine diseases, is a dominantly inherited movement disorder characterized by formation of cytosolic and nuclear inclusion bodies and progressive neurodegeneration.
Mathias Bähr, Pawel Kermer, Sebastian Deeg, Christoph P. Dohm, Kamila Sroka, Jan Liman   +5 more
doaj   +1 more source

The Unfolded Protein Response and its potential role in Huntington's disease [PDF]

, 2012
Huntington's disease (HD) is a progressive, neurodegenerative disease with fatal outcome. Although the disease-causing gene (huntingtin) has been known for some time, the exact cause of neuronal cell death is still unknown.
Kamesh Ayasolla, Matthias E. Futschik, Ravi K. R. Kalathur   +2 more
core   +2 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 predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor

BMC Neuroscience, 2002
Background Huntington's disease (HD) pathogenesis is due to an expanded polyglutamine tract in huntingtin, but the specificity of neuronal loss compared with other polyglutamine disorders also implies a role for the protein's unknown inherent function ...
Gusella James F, Takano Hiroki
doaj   +1 more source

Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression

Scientific Reports, 2015
Quantitation of huntingtin protein in the brain is needed, both as a marker of Huntington disease (HD) progression and for use in clinical gene silencing trials.
Amber L. Southwell, Stephen E.P. Smith, T. Davis, N. Caron, Erika B Villanueva, Yuanyun Xie, Jennifer A. Collins, Min Li Ye, Aaron Sturrock, B. Leavitt, A. Schrum, Michael R. Hayden   +11 more
semanticscholar   +1 more source

A huntingtin peptide inhibits polyQ-huntingtin associated defects. [PDF]

PLoS ONE, 2013
Huntington's disease (HD) is caused by the abnormal expansion of the polyglutamine tract in the human Huntingtin protein (polyQ-hHtt). Although this mutation behaves dominantly, huntingtin loss of function also contributes to HD pathogenesis.
Yoan Arribat, Nathalie Bonneaud, Yasmina Talmat-Amar, Sophie Layalle, Marie-Laure Parmentier, Florence Maschat   +5 more
doaj   +1 more source

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
core   +4 more sources

Altered microRNA expression in animal models of Huntington’s disease and potential therapeutic strategies

Neural Regeneration Research, 2021
A review of recent animal models of Huntington’s disease showed many microRNAs had altered expression levels in the striatum and cerebral cortex, and which were mostly downregulated.
Bridget Martinez, Philip V Peplow
doaj   +1 more source

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
core   +4 more sources

Huntingtin and Its Partner Huntingtin-Associated Protein 40: Structural and Functional Considerations in Health and Disease

Journal of Huntington's Disease, 2022
Since the discovery of the mutation causing Huntington’s disease (HD) in 1993, it has been debated whether an expanded polyglutamine (polyQ) stretch affects the properties of the huntingtin (HTT) protein and thus contributes to the pathological mechanisms responsible for HD.
Seefelder, Manuel, Klein, Fabrice A.C., Landwehrmeyer, Bernhard, Fernández Busnadiego, Rubén, Kochanek, Stefan   +4 more
openaire   +3 more sources