AAA proteases of mitochondria: quality control of membrane proteins and regulatory functions during mitochondrial biogenesis. (August 2001)

• Record Type: Journal Article
• Authors: Langer, T.; Käser, M.; Klanner, C.; Leonhard, K.
• Is Part Of: Biochemical Society transactions. Volume 29:Number 4(2001)
• Page Start: 431
• Page End: 436
• Publication Date: 2001-08
• Subjects: AAA protease -- ABC transporter -- prohibitin -- proteolysis; ER, endoplasmic reticulum; Biochemistry -- Congresses; 572
• DOI: 10.1042/bst0290431
• Languages: English
• ISSNs: 0300-5127

Abstract

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.