期刊
JOURNAL OF HUNTINGTONS DISEASE
卷 2, 期 2, 页码 149-157出版社
IOS PRESS
DOI: 10.3233/JHD-130052
关键词
Huntington's disease; huntingtin; polyglutamine expansion; autophagy
资金
- National Natural Science Foundation of China [30600197]
- National Basic Science Key Project (973 project) [CB51003]
- Priority Academic Program Development of Jiangsu Higher Education Institutes (PAPD)
Autophagy is a degradation pathway for long-lived cytoplasmic proteins, protein complexes, or damaged organelles. The accumulation and aggregation of misfolded proteins are hallmarks of several neurodegenerative diseases. Many researchers have reported that autophagy degrades disease-causing misfolded and aggregated proteins, including mutant huntingtin (Htt) in Huntington's disease, mutant synuclein in familial Parkingson's disease, mutant Cu, Zn(-)Superoxide dismutase (SOD1) in familial amyotrophic lateral sclerosis. In this review, we will bring up new evidence to elucidate the involvement of autophagy in degradation of mutant Htt, discuss the mechanisms regulating the degradation of mutant Htt by autophagy and the therapeutic effects of drugs that enhance autophagy to improve clearance of mutant Htt. We propose that enhancement of autophagy by drugs may be a strategy to treat or retard progression of Huntington's disease.
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