Proteolytic control of mitochondrial function and morphogenesis

被引:97
作者
Anand, Ruchika [1 ]
Langer, Thomas [1 ,2 ]
Baker, Michael James [1 ]
机构
[1] Univ Cologne, Inst Genet, Ctr Mol Med CMMC, Cologne Excellence Cluster Cellular Stress Respon, D-50674 Cologne, Germany
[2] Max Planck Inst Biol Aging, D-50931 Cologne, Germany
来源
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH | 2013年 / 1833卷 / 01期
基金
欧洲研究理事会;
关键词
Mitochondria; Quality control; Mitophagy; Mitochondrial dynamics; AAA protease; Lon protease; M-AAA PROTEASE; DYNAMIN-RELATED PROTEIN-1; END RULE PATHWAY; QUALITY-CONTROL; LON PROTEASE; DEPENDENT DEGRADATION; SELECTIVE DEGRADATION; SPASTIC PARAPLEGIA; MEMBRANE-PROTEINS; OMA1; PROTEASE;
D O I
10.1016/j.bbamcr.2012.06.025
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
070307 [化学生物学]; 071010 [生物化学与分子生物学];
摘要
Mitochondrial proteostasis depends on a hierarchical system of tightly controlled quality surveillance mechanisms. Proteases within mitochondria take center stage in this network. They eliminate misfolded and damaged proteins and ensure the biogenesis and morphogenesis of mitochondria by processing or degrading short-lived regulatory proteins. Mitochondrial gene expression, the mitochondrial phospholipid metabolism and the fusion of mitochondrial membranes are under proteolytic control. Furthermore, in response to stress and mitochondria( dysfunction, proteolysis inhibits fusion and facilitates mitophagy and apoptosis. Defining these versatile activities of mitochondrial proteases will be pivotal for understanding the pathogenesis of various neurodegenerative disorders associated with defective mitochondria-associated proteolysis. This article is part of a Special Issue entitled: Mitochondrial dynamics and physiology. (c) 2012 Elsevier B.V. All rights reserved.
引用
收藏
页码:195 / 204
页数:10
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