T. Langer et al., AAA proteases of mitochondria: quality control of membrane proteins and regulatory functions during mitochondrial biogenesis, BIOCH SOC T, 29, 2001, pp. 431-436
An ubiquitous and conserved proteolytic regulates the stability of mitochon
drial inner membrane proteins. Two AAA proteases,vith catalytic sites at op
posite membrane surfaces form a membrane-integrated quality control system
and exert crucial functions during the biogenesis of mitochondria. Their ac
tivity is modulated by another membrane-protein complex that is composed of
prohibitins. Peptides generated upon proteolysis in the matrix space are t
ransported across the inner membrane by an ATP-binding cassette transporter
. The function of these conserved components is discussed in the present re
view.