Mitochondrial respiratory chain complexes assembly protein AFG3; Mitochondrial inner membrane m-AAA protease component; mediates degradation of misfolded or unassembled proteins; also required for correct assembly of mitochondrial enzyme complexes; involved in cytoplasmic mRNA translation and aging; expression of human homolog AFG3L2 can complement yeast yta12 afg3 double mutant; In the N-terminal section; belongs to the AAA ATPase family.

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.

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.

Mitochondrial translation elongation factor Tu (EF-Tu); involved in fundamental pathway of mtDNA homeostasis; comprises both GTPase and guanine nucleotide exchange factor activities, while these activities are found in separate proteins in S. pombe and humans; rare mutations in human mitochondrial elongation factor Tu (EFTu) associated with severe lactic acidosis, rapidly progressive fatal encephalopathy, severe infantile macrocystic leukodystrophy with micropolygyria.

Mitochondrial outer membrane protein porin 2; Putative mitochondrial porin (voltage-dependent anion channel); not required for mitochondrial membrane permeability or mitochondrial osmotic stability; POR2 has a paralog, POR1, that arose from the whole genome duplication.

Outer mitochondrial membrane GTPase, subunit of the ERMES complex; potential regulatory subunit of the ERMES complex that links the ER to mitochondria and may promote inter-organellar calcium and phospholipid exchange as well as coordinating mitochondrial DNA replication and growth; cells lacking Gem1p contain collapsed, globular, or grape-like mitochondria; ortholog of metazoan Miro GTPases.

Protein involved in mitochondrial fission and peroxisome abundance; may have a distinct role in tethering protein aggregates to mitochondria in order to retain them in the mother cell; required for localization of Dnm1p and Mdv1p during mitochondrial division; mediates ethanol-induced apoptosis and ethanol-induced mitochondrial fragmentation; Belongs to the FIS1 family.

Rhomboid protein 1, mitochondrial; Mitochondrial serine protease; required for the processing of various mitochondrial proteins and maintenance of mitochondrial DNA and morphology; belongs to the rhomboid-GlpG superfamily of intramembrane peptidases.

Essential component of the TIM23 complex; acts as receptor for the translocase of the inner mitochondrial membrane (TIM23) complex guiding incoming precursors from the TOM complex; may control the gating of the Tim23p-Tim17p channel.