title: AAA proteases of mitochondria: quality control of membrane proteins and regulatory functions during mitochondrial biogenesis.
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.
authors.affiliation: Institut für Genetik, Universität zu Köln, Zülpicher Str. 47, 50674 Köln, Germany. thomas.langer@uni-koeln.de
authors.forename: T
authors.initials: T
authors.lastname: Langer
authors.forename: M
authors.initials: M
authors.lastname: Käser
authors.forename: C
authors.initials: C
authors.lastname: Klanner
authors.forename: K
authors.initials: K
authors.lastname: Leonhard
mesh_terms: 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
chemical_list: 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