期刊
AUTOPHAGY
卷 9, 期 2, 页码 234-235出版社
LANDES BIOSCIENCE
DOI: 10.4161/auto.22501
关键词
Niemann-Pick; autophagy; lysosome; lipid storage; cholesterol; cathepsin; neurodegeneration
类别
资金
- NINDS NIH HHS [F30 NS065662, R01 NS063967, F31 NS065662] Funding Source: Medline
Alterations in macroautophagy (hereafter referred to as autophagy) are a common feature of lysosomal storage disorders, and have been hypothesized to play a major role in the pathogenesis of these diseases. We have recently reported multiple defects in autophagy contributing to the lysosomal storage disorder Niemann-Pick type C (NPC). These include increased formation of autophagosomes, slowed turnover of autophagosomes secondary to impaired lysosomal proteolysis, and delivery of stored lipids to the lysosome via autophagy. The study summarized here describes novel methods for the interrogation of individual stages of the autophagic pathway, and suggests mechanisms by which lipid storage may result in broader lysosomal dysfunction.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据