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

T Langer, M Käser, C Klanner… - Biochemical Society …, 2001 - portlandpress.com
T Langer, M Käser, C Klanner, K Leonhard
Biochemical Society Transactions, 2001portlandpress.com
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 …
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.
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