Focus Quality Control
- The EMBO Journal (2008) 27, 306 - 314
- doi:10.1038/sj.emboj.7601972
Subject Categories:
Quality control of mitochondria: protection against neurodegeneration and ageing
Takashi Tatsuta1 and Thomas Langer1
- Institute for Genetics and Centre for Molecular Medicine (CMMC), University of Cologne, Cologne, Germany
Correspondence to:
Thomas Langer, Institut für Genetik, Universität zu Köln, Zülpicher Str. 47, Köln 50674, Germany. Tel.: +49 221 470 4876; Fax: +49 221 470 6749; E-mail: Thomas.Langer@uni-koeln.de
Received 12 November 2007; Accepted 4 December 2007
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.
Keywords:
- mitochondria,
- mitochondrial dynamics,
- mitochondrial proteases,
- mitophagy,
- neurodegeneration
