ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU . Required for paraplegin (SPG7) maturation . After its cleavage by mitochondrial-processing peptidase (MPP), it converts paraplegin into a proteolytically active mature form (By similarity). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (, ). Involved in the regulation of OMA1-dependent processing of OPA1 (, ). Contributes to the proteolytic degradation of GHITM upon hyperpolarization of mitochondria . Progressive GHITM degradation upon persistent hyperpolarization leads to respiratory complex I degradation and broad reshaping of the mitochondrial proteome by AFG3L2 .
Subcellular locations: Mitochondrion, Mitochondrion inner membrane
Ubiquitous. Highly expressed in the cerebellar Purkinje cells.