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EMBO J. 2008 Jan 23;27(2):306-14. doi: 10.1038/sj.emboj.7601972.

Quality control of mitochondria: protection against neurodegeneration and ageing.

Author information

1
Institute for Genetics and Centre for Molecular Medicine, University of Cologne, Cologne, Germany.

Abstract

Dysfunction of mitochondria has severe cellular consequences and is linked to ageing and neurodegeneration in human. Several surveillance strategies have evolved that limit mitochondrial damage and ensure cellular integrity. Intraorganellar proteases conduct protein quality control and exert regulatory functions, membrane fusion and fission allow mitochondrial content mixing within a cell, and the autophagic degradation of severely damaged mitochondria protects against apoptosis. Here, we will summarize the current knowledge on these surveillance strategies and their role in human disease.

PMID:
18216873
PMCID:
PMC2234350
DOI:
10.1038/sj.emboj.7601972
[Indexed for MEDLINE]
Free PMC Article

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