Lowering Mutant Huntingtin Levels and Toxicity: Autophagy-Endolysosome Pathways in Huntington's Disease

被引：32
作者：
Valionyte, Evelina [1 ,2 ]
Yang, Yi [1 ,2 ]
Roberts, Sheridan L. [1 ,2 ]
Kelly, Jack [1 ,2 ]
Lu, Boxun [3 ]
Luo, Shouqing [1 ,2 ]
机构：
[1] Univ Plymouth, Peninsula Sch Med, Inst Translat & Stratified Med, Res Way, Plymouth PL6 8BU, Devon, England
[2] Univ Plymouth, Peninsula Sch Dent, Inst Translat & Stratified Med, Res Way, Plymouth PL6 8BU, Devon, England
[3] Fudan Univ, Collaborat Innovat Ctr Brain Sci, Sch Life Sci, State Key Lab Med Neurobiol, Shanghai 200438, Peoples R China
来源：
JOURNAL OF MOLECULAR BIOLOGY | 2020年 / 432卷 / 08期
基金：
中国国家自然科学基金; 英国医学研究理事会;
关键词：
Autophagy; Selective autophagy; Huntington's disease; Huntingtin; Neu rodegeneration; INCLUSION-BODY FORMATION; SELECTIVE AUTOPHAGY; MOUSE MODEL; POLYGLUTAMINE EXPANSIONS; MOLECULAR-MECHANISMS; TRINUCLEOTIDE REPEAT; ACTIVATES AUTOPHAGY; RECEPTOR OPTINEURIN; MEDIATED AUTOPHAGY; NUCLEAR INCLUSIONS;
D O I：
10.1016/j.jmb.2019.11.012
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
Huntington's disease (HD) is a monogenetic neurodegenerative disease, which serves as a model of neurodegeneration with protein aggregation. Autophagy has been suggested to possess a great value to tackle protein aggregation toxicity and neurodegenerative diseases. Current studies suggest that autophagyendolysosomal pathways are critical for HD pathology. Here we review recent advancement in the studies of autophagy and selective autophagy relating HD. Restoration of autophagy flux and enhancement of selective removal of mutant huntingtin/disease-causing protein would be effective approaches towards tackling HD as well as other similar neurodegenerative disorders. (C) 2019 Elsevier Ltd. All rights reserved.
引用
收藏
页码：2673 / 2691
页数：19
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