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Biochem Soc Trans. 2001 Aug;29(Pt 4):431-6.

AAA proteases of mitochondria: quality control of membrane proteins and regulatory functions during mitochondrial biogenesis.

Author information

1
Institut für Genetik, Universität zu Köln, Zülpicher Str. 47, 50674 Köln, Germany. thomas.langer@uni-koeln.de

Abstract

An ubiquitous and conserved proteolytic system regulates the stability of mitochondrial inner membrane proteins. Two AAA proteases with catalytic sites at opposite membrane surfaces form a membrane-integrated quality control system and exert crucial functions during the biogenesis of mitochondria. Their activity is modulated by another membrane-protein complex that is composed of prohibitins. Peptides generated upon proteolysis in the matrix space are transported across the inner membrane by an ATP-binding cassette transporter. The function of these conserved components is discussed in the present review.

PMID:
11498003
[Indexed for MEDLINE]

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