AAA proteases of mitochondria: quality control of membrane proteins and regulatory functions during mitochondrial biogenesis.
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.
Langer
T
T
Institut für Genetik, Universität zu Köln, Zülpicher Str. 47, 50674 Köln, Germany. thomas.langer@uni-koeln.de
Käser
M
M
Klanner
C
C
Leonhard
K
K
D002736:Chloroplasts / Q000201:enzymology; D010450:Endopeptidases / Q000378:metabolism*; D008565:Membrane Proteins / Q000378:metabolism*; D008928:Mitochondria / Q000201:enzymology / Q000502:physiology*; D008954:Models, Biological; D010447:Peptide Hydrolases / Q000378:metabolism; D000091402:Prohibitins; D046988:Proteasome Endopeptidase Complex; D021122:Protein Subunits; D011506:Proteins / Q000378:metabolism; D012097:Repressor Proteins; D012441:Saccharomyces cerevisiae / Q000201:enzymology
D008565:Membrane Proteins; D000091402:Prohibitins; D021122:Protein Subunits; D011506:Proteins; D012097:Repressor Proteins; D010450:Endopeptidases; D010447:Peptide Hydrolases; D046988:Proteasome Endopeptidase Complex; C061553:ATP dependent 26S protease