Autophagy in neurodegeneration and development

被引：104

作者：

Winslow, Ashley R. ^{[1
]}

Rubinsztein, David C. ^{[1
]}

机构：

[1] Addenbrookes Hosp, Cambridge Inst Med Res, Dept Med Genet, Cambridge CB2 0XY, England

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE | 2008年 / 1782卷 / 12期

基金：

英国惠康基金; 英国医学研究理事会;

关键词：

Autophagy; Neurodegeneration; Development; Endosomal sorting complexes required for transport (ESCRT); Lysosomal storage disorder (LSD); Dynein;

D O I：

10.1016/j.bbadis.2008.06.010

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：

Efficient protein turnover is essential for the maintenance of cellular health. Here we review how autophagy has fundamental functions in cellular homeostasis and possible uses as a therapeutic strategy for neurodegenerative diseases associated with intracytosolic aggregate formation, like Huntington's disease (HD). Drugs like rapamycin, that induce autophagy, increase the clearance of mutant huntingtin fragments and ameliorate the pathology in cell and animal models of HD and related conditions. In Drosophila, the beneficial effects of rapamycin in diseases related to HD are autophagy-dependent. We will also discuss the importance of autophagy in early stages of development and its possible contribution as a secondary disease mechanism in forms of fronto-temporal dementias, motor neuron disease, and lysosomal storage disorders. (C) 2008 Elsevier B.V. All rights reserved.

引用

页码：723 / 729

页数：7

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