Mitochondrial respiratory chain complexes assembly protein YTA12; Mitochondrial inner membrane m-AAA protease component; mediates degradation of misfolded or unassembled proteins; also required for correct assembly of mitochondrial enzyme complexes; overexpression of human AFG3L2 complements respiratory defect of yeast afg3 yta12 double null mutation, but overexpression of disease-associated AFG3L2 variants does not; expression of both human SPG7 (paraplegin) and AFG3L2 complements yeast yta12 afg3 double mutation; In the N-terminal section; belongs to the AAA ATPase family.

Prohibitin-2; Subunit of the prohibitin complex (Phb1p-Phb2p); prohibitin is a 1.2 MDa ring-shaped inner mitochondrial membrane chaperone that stabilizes newly synthesized proteins; determinant of replicative life span; involved in mitochondrial segregation; prohibitin deficiency induces a mitochondrial unfolded protein response (mtUPR).

ATP-dependent Lon protease; involved in degradation of misfolded proteins in mitochondria; required for biogenesis and maintenance of mitochondria; Belongs to the peptidase S16 family.

Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; Mic60p is also involved in import of intermembrane space (IMS) proteins, probably by positioning Mia40p relative to the TOM complex to receive incoming proteins; ortholog of mammalian mitofilin.

Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress.

Metalloendopeptidase of the mitochondrial inner membrane; important for adaptive responses to various homeostatic insults, preservation of normal mitochondrial function under damage-eliciting conditions, and stability of respiratory supercomplexes; involved in turnover of membrane-embedded proteins; mediates degradation of Cox1p in coa2 mutant cells; zebrafish ortholog has a role in organ development and mouse ortholog is also required for respiratory supercomplex stability.

Catalytic subunit of i-AAA protease complex; complex is located in mitochondrial inner membrane; responsible for degradation of unfolded or misfolded mitochondrial gene products; serves as nonconventional translocation motor to pull PNPase into intermembrane space; also has role in intermembrane space protein folding; mutation causes elevated rate of mitochondrial turnover; human homolog YME1L1 can complement yeast null mutant; In the C-terminal section; belongs to the peptidase M41 family.

Subunit of the mitochondrial (mt) i-AAA protease supercomplex; i-AAA degrades misfolded mitochondrial proteins; forms a subcomplex with Mgr1p that binds to substrates to facilitate proteolysis; required for growth of cells lacking mtDNA.