Table 2.

Mitochondrial Proteases

name
in yeast	in mammals	description	localization
Atp23	XRCC6BP1	metalloprotease of the IM; participates in degradation of Ups1	IMS
Icp55	XPNPEP3	aminopeptidase that removes unstable N-terminal residues exposed by MPP	matrix
Imp1; Imp2	IMMP1L; IMMP2L	form the IM peptidase complex; required for maturation of certain IMS proteins	IM
Mas1; Mas2	MPPα; MPPβ	subunits of heterodimeric mitochondrial processing peptidase (MPP); removes N-terminal mitochondrial targeting sequence	matrix
Oct1	MIPEP	mitochondrial intermediate peptidase; removes octapeptides from selected imported proteins with an unstable N-terminal residue exposed by MPP	matrix
Oma1	OMA1	metalloendopeptidase; in mammals, involved in stress-regulated cleavage of OPA1; in yeast, has a role in turnover of Psd1ts	IM
Pcp1	PARL	serine protease and rhomboid intramembrane protease family member; in yeast, cleaves Mgm1; in mammals, cleaves STARD7 in TIM23-dependent and -independent manners	IM
Pim1	LONP1	member of the AAA+ and serine peptidase families; important for QC in the matrix	matrix
Yme1	YME1L	catalytic subunit of i-AAA protease; degrades unfolded/misfolded mitochondrial proteins in the IMS; substrates include Ups1, Ups2, STARD7, certain BTHS mutant tafazzins, and Psd1ts	IM
Yta10; Yta12	AFG3L1; AFG3L2; SPG7	subunits of homo- or hetero-oligomeric m-AAA protease; mediate degradation of misfolded or unassembled proteins in the matrix	IM