自噬
神经退行性变
亨廷顿蛋白
生物
细胞生物学
神经保护
神经科学
亨廷顿病
蛋白质聚集
机制(生物学)
疾病
医学
突变体
遗传学
基因
细胞凋亡
病理
哲学
认识论
作者
Aurora Scrivo,Mathieu Bourdenx,Olatz Pampliega,Ana María Cuervo
标识
DOI:10.1016/s1474-4422(18)30238-2
摘要
Cells rely on surveillance systems such as autophagy to handle protein alterations and organelle damage. Dysfunctional autophagy, an evolutionarily conserved cellular mechanism for degradation of intracellular components in lysosomes, frequently leads to neurodegeneration. The neuroprotective effect of autophagy stems from its ability to eliminate pathogenic forms of proteins such as α-synuclein or tau. However, the same pathogenic proteins often affect different types and steps of the autophagic process. Furthermore, genetic studies have shown that some proteins related to neurodegeneration, such as huntingtin, participate in autophagy as one of their physiological functions. This complex interplay between autophagy and neurodegeneration suggests that targeting autophagy as a whole might have limited applicability in neurodegenerative diseases, and that future efforts should focus instead on targeting specific types and steps of the autophagic process. This change of strategy in the modulation of autophagy might hold promise for future disease-modifying therapies for patients with neurodegenerative disorders.
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