期刊
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
卷 1837, 期 4, 页码 451-460出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbabio.2013.11.010
关键词
Mitochondrion; Mitochondria fusion/fission; Autophagy; Ubiquitylation; Neurodegeneration; Mitochondria Quality Control
资金
- Italian Ministry of Health [GR-2009-1606827]
- AIRC Program MyFAG [MFAG-12120]
- AIRC [IG2010, IG2012]
- FISM
- Italian Ministry of University and Research
- Italian Ministry of Health
Mitochondria are double-membraned highly dynamic organelles; the shape, location and function of which are determined by a constant balance between opposing fusion and fission events. A fine modulation of mitochondrial structure is crucial for their correct functionality and for many physiological cell processes, the status of these organelles, being thus a key aspect in a cell's fate. Indeed, the homeostasis of mitochondria needs to be highly regulated for the above mentioned reasons, and since a) they are the major source of energy; b) they participate in various signaling pathways; albeit at the same time c) they are also the major source of reactive oxygen species (ROS, the main damaging detrimental players for all cell components). Elaborate mechanisms of mitochondrial quality control have evolved for maintaining a functional mitochondrial network and avoiding cell damage. The first mechanism is the removal of damaged mitochondrial proteins within the organelle via chaperones and protease; the second is the cytosolic ubiquitin-proteasome system (UPS), able to eliminate proteins embedded in the outer mitochondrial membrane; the third is the removal of the entire mitochondria through mitophagy, in the case of extensive organelle damage and dysfunction. In this review, we provide an overview of these mitochondria stability and quality control mechanisms, highlighting mitophagy, and emphasizing the central role of mitochondrial dynamics in this context. This article is part of a Special Issue entitled: Dynamic and ultrastructure of bioenergetic membranes and their components. (C) 2013 The Authors. Published by Elsevier B.V. All rights reserved.
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