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AMT-130 gene therapy: a promising disease-modifying approach for Huntington's disease. [PDF]
Mwape C +7 more
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Ubiquitin ligase Nedd4 regulates the abundance and toxicity of mutant huntingtin. [PDF]
Jeong H +9 more
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Huntingtin: A Protein with a Peculiar Solvent Accessible Surface
Taking advantage of the last cryogenic electron microscopy structure of human huntingtin, we explored with computational methods its physicochemical properties, focusing on the solvent accessible surface of the protein and highlighting a quite interesting mix of hydrophobic and hydrophilic patterns, with the prevalence of the latter ones.
Giulia Babbi +2 more
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Autophagy is one of the well-known pathways to accelerate the clearance of protein aggregates, which contributes to the therapy of neurodegenerative diseases.
Pengfei Wei +2 more
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Huntington disease and the huntingtin protein.
Progress in Molecular Biology and Translational Science, 2012Huntington disease (HD) is a devastating neurodegenerative disease that derives from CAG repeat expansion in the huntingtin gene. The clinical syndrome consists of progressive personality changes, movement disorder, and dementia and can develop in children and adults.
Zhiqiang Zheng, M. Diamond
semanticscholar +3 more sources
Arfaptin 2 regulates the aggregation of mutant huntingtin protein
Huntington's disease (HD) is an inherited neurodegenerative disorder. Here we demonstrate that expression of arfaptin 2/POR1 (partner of Rac1) in cultured cells induces the formation of pericentriolar and nuclear aggregates, which morphologically ...
P. Peters +8 more
semanticscholar +2 more sources
Huntingtin-protein interactions and the pathogenesis of Huntington's disease.
Trends in Genetics, 2004At least nine inherited neurodegenerative diseases share a polyglutamine expansion in their respective disease proteins. These diseases show distinct neuropathological changes, suggesting that protein environment and protein-protein interactions play an important role in the specific neuropathology.
Shihua Li, Xiao-Jiang Li
semanticscholar +3 more sources
Cellular and Molecular Life Sciences, 2022
Huntingtin-associated protein 1 (HAP1) is the first identified protein whose function is affected by its abnormal interaction with mutant huntingtin (mHTT), which causes Huntington disease. However, the expression patterns of Hap1 and Htt in the rodent brain are not correlated.
Xingxing Chen +15 more
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Huntingtin-associated protein 1 (HAP1) is the first identified protein whose function is affected by its abnormal interaction with mutant huntingtin (mHTT), which causes Huntington disease. However, the expression patterns of Hap1 and Htt in the rodent brain are not correlated.
Xingxing Chen +15 more
openaire +2 more sources
Huntingtin protein: A new option for fixing the Huntington's disease countdown clock
Neuropharmacology, 2018&NA; Huntington's disease is a dreadful, incurable disorder. It springs from the autosomal dominant mutation in the first exon of the HTT gene, which encodes for the huntingtin protein (HTT) and results in progressive neurodegeneration. Thus far, all the
M. Caterino +5 more
semanticscholar +1 more source

