Results 121 to 130 of about 6,546,213 (228)

Compounds blocking mutant huntingtin toxicity identified using a Huntington's disease neuronal cell model

Neurobiology of Disease, 2005
Neuronal cell death in HD is believed to be largely a dominant cell-autonomous effect of the mutant huntingtin protein. We previously developed an inducible PC12 cell model which expresses an N-terminal huntingtin fragment with an expanded poly Q repeat (
Wenfei Wang, Wenzhen Duan, Shuichi Igarashi, Hokuto Morita, Masayuki Nakamura, Christopher A. Ross   +5 more
doaj   +1 more source

ALTERED EXPRESSION AND FUNCTIONALITY OF A2A ADENOSINE RECEPTORS IN HUNTINGTON’S DISEASE AND OTHER POLYGLUTAMINE DISORDERS

, 2009
Several studies have suggested the possible involvement of A2A adenosine receptors in the pathogenesis of neuronal disorders, including Huntington’s disease.
Vincenzi, Fabrizio
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Reduction of mutant huntingtin accumulation and toxicity by lysosomal cathepsins D and B in neurons

Molecular Neurodegeneration, 2011
Background Huntington's disease is caused by aggregation of mutant huntingtin (mHtt) protein containing more than a 36 polyQ repeat. Upregulation of macroautophagy was suggested as a neuroprotective strategy to degrade mutant huntingtin.
Ouyang Xiaosen, Liang Qiuli, Schneider Lonnie, Zhang Jianhua   +3 more
doaj   +1 more source

The role of glia in protein misfolding diseases

, 2010
The astrocytes, oligodendrocytes and microglia make up a significant proportion of the cells of the CNS. In recent years, there has been a burgeoning interest in the role of glial cells, in neurodegenerative disease.
Samson, Ben
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Disordered Nanostructure in Huntingtin Interacting Protein K Acts as a Stabilizing Switch To Prevent Protein Aggregation

, 2018
Protein misfolding due to mutation(s) and/or generation of unstable intermediate state(s) can be the cause of aberrant aggregations, leading to cellular degeneration. While molecular signatures like amyloidogenic regions cause aggregation, other features
Debasish Kumar Ghosh (4849174), Akash Ranjan (4953), Ajit Roy (1423147)   +2 more
core   +2 more sources

Neferine Attenuates the Protein Level and Toxicity of Mutant Huntingtin in PC-12 Cells via Induction of Autophagy

Molecules, 2015
Mutant huntingtin aggregation is highly associated with the pathogenesis of Huntington’s disease, an adult-onset autosomal dominant disorder, which leads to a loss of motor control and decline in cognitive function.
V. Wong, Anguo Wu, J. Wang, Liang Liu, B. Law   +4 more
semanticscholar   +1 more source

p53 tumor suppressor protein regulates the levels of huntingtin gene expression

, 2006
The p53 protein is a transcription factor that integrates various cellular stress signals. The accumulation of the mutant huntingtin protein with an expanded polyglutamine tract plays a central role in the pathology of human Huntington's disease.
Feng, Z., Hoh, J., Lowe, S. W., Levine, A. J., Zupnick, A., De Stanchina, E., Prives, C., Jin, S.   +7 more
core   +1 more source

Development of novel bioassays to detect soluble and aggregated Huntingtin proteins on three technology platforms [PDF]

, 2021
Huntington’s disease is caused by a CAG / polyglutamine repeat expansion. Mutated CAG repeats undergo somatic instability, resulting in tracts of several hundred CAGs in the brain; and genetic modifiers of Huntington’s disease have indicated that somatic
Taxy, BA, McLarnon, S, Jean, A, McAteer, SJ, Zhang, C, Howland, D, Landles, C, Bates, GP, Milton, RE, Duan, W, Osborne, GF   +10 more
core  

The huntingtin protein in Huntington disease

, 2017
Huntingtin disease (HD) is an autosomal dominant inheritable disease that mainly affects the brain. HD is caused by expansion of a CAG repeat within exon 1 of the huntingtin gene giving rise to the expression of a mutant huntingtin protein with an ...
Schut, M.H.
core   +1 more source

INVESTIGATION OF THE HUNTINGTIN-HAP40 INTERACTION IN HUNTINGTON’S DISEASE [PDF]

, 2014
Huntington’s disease (HD) is a neurodegenerative disorder caused by the expansion of a polyglutamine tract in the huntingtin protein. The huntingtin protein has many roles in vesicular and endocytic trafficking, which can be modified in HD cells.
Williamson, Jennifer
core