Table 1.

Intrinsic mitochondrial proteases and functions.

Category	Symbol	Class	Localization	Functions	Reference(s)
	CLPP	Ser	Matrix	Protein quality control transcription/Translation ribosome assembly	25,95,96
ATP-dependent proteases	LONP1	Ser	Matrix	Protein quality control Mitochondrial biogenesis mtDNA maintainence mtDNA replication Adaptation to hypoxia	25,96–98
	AFG3L2 AFG3L2/SPG7	Metallo	Matrix/IM	Protein quality control Mitochondrial biogenesis Ribosome assembly MCU assembly	15,25,99
	YME1L (FTSH1)	Metallo	IM/IMS	Protein quality control Mitochondrial biogenesis Protein import Lipid trafficking Mitochondrial dynamics	17,100,101
	ATP23	Metallo	IMS	Protein quality control Protein maturation F1FO-ATP synthase assembly	102,103
	IMMP1L IMMP2L	Ser	IM/IMS	Protein maturation Apoptosis/senescence	104,105
	METAP1D	Metallo	Matrix	Protein import and activation	15,25,106
Processing peptidases	MIP	Metallo	Matrix	Coenzyme Q biosynthesis Complex III and IV activity Protein import and activation	15,107,108
	OMA1	Metallo	IMS/IM	Mitochondrial dynamics mitophagy and apoptosis	17 25
	PARL	Ser	IM	Mitophagy and apoptosis Coenzyme Q biosynthesis Complex III assembly Lipid trafficking	109 110 111,112
	PMPCB	Metallo	Matrix	Protein maturation	15,113
	XPNPEP3	Metallo	Matrix	Protein import and activation Protein stability	114
Oligopeptidases	MEP	Metallo	IMS	Protein quality control	15 25
	PITRM1	Metallo	Matrix	Protein quality control	15 25
Other mitochondrial proteases	HTRA2 (OMI)	Ser	IMS	Protein quality control mitophagy and apoptosis Stress signaling Cristae structure maintenance	115,116 18
	LACTB	Ser	IMS	Mitochondrial biogenesis PE metabolism	117 25

IM inner membrane, IMS intermembrane space, MCU mitochondrial Ca2+ uniporter, PE phosphatidylethanolamine.