Journal
JOURNAL OF BIOCHEMISTRY
Volume 146, Issue 4, Pages 455-461Publisher
OXFORD UNIV PRESS
DOI: 10.1093/jb/mvp122
Keywords
mitochondria; ATP-dependent proteases; chaperones; mitochondrial unfolded protein response; protein aggregation
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Funding
- Deutsche Forschungsgemeinschaft [SFB635]
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Mitochondria are crucial for both life and death of eukaryotic cells. Compromised mitochondrial integrity has severe cellular consequences and is linked to senescence and neurodegenerative disorders in humans. To maintain the functionality of proteins in mitochondria, quality-control mechanisms including signal transduction pathways counteracting mitochondrial stress have evolved. A network of molecular chaperones and proteases monitors protein integrity and prevents accumulation of damaged proteins. In this review, the current knowledge of elaborate defence strategies within mitochondria is summarized.
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