自噬
溶酶体
ATG16L1
下调和上调
细胞生物学
生物
亨廷顿蛋白
疾病
蛋白质降解
袋3
肌萎缩侧索硬化
亨廷顿病
癌症研究
医学
遗传学
生物化学
细胞凋亡
病理
基因
酶
作者
Alvin Djajadikerta,Swati Keshri,Mariana Pavel,Ryan Prestil,Laura Ryan,David C. Rubinsztein
标识
DOI:10.1016/j.jmb.2019.12.035
摘要
Autophagy is a major, conserved cellular pathway by which cells deliver cytoplasmic contents to lysosomes for degradation. Genetic studies have revealed extensive links between autophagy and neurodegenerative disease, and disruptions to autophagy may contribute to pathology in some cases. Autophagy degrades many of the toxic, aggregate-prone proteins responsible for such diseases, including mutant huntingtin (mHTT), alpha-synuclein (α-syn), tau, and others, raising the possibility that autophagy upregulation may help to reduce levels of toxic protein species, and thereby alleviate disease. This review examines autophagy induction as a potential therapy in several neurodegenerative diseases—Alzheimer’s disease, Parkinson’s disease, polyglutamine diseases, and amyotrophic lateral sclerosis (ALS). Evidence in cells and in vivo demonstrates promising results in many disease models, in which autophagy upregulation is able to reduce the levels of toxic proteins, ameliorate signs of disease, and delay disease progression. However, the effective therapeutic use of autophagy induction requires detailed knowledge of how the disease affects the autophagy-lysosome pathway, as activating autophagy when the pathway cannot go to completion (e.g., when lysosomal degradation is impaired) may instead exacerbate disease in some cases. Investigating the interactions between autophagy and disease pathogenesis is thus a critical area for further research.
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