Heat shock protein 78, mitochondrial; Oligomeric mitochondrial matrix chaperone; cooperates with Ssc1p in mitochondrial thermotolerance after heat shock; able to prevent the aggregation of misfolded proteins as well as resolubilize protein aggregates.

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 glycine transporter; required for the transport of glycine into mitochondria for initiation of heme biosynthesis, with YMC1 acting as a secondary transporter; homolog of human SLC25A38, a mitochondrial glycine transporter associated with nonsyndromic autosomal recessive congenital sideroblastic anemia; human SLC25A38 can complement the heme deficiency associated with the null mutant; GFP-fusion protein is induced in response to the DNA-damaging agent MMS.

Probable glutathione-independent glyoxalase HSP33; Possible chaperone and cysteine protease; required for transcriptional reprogramming during the diauxic shift and for survival in stationary phase; similar to E. coli Hsp31 and S. cerevisiae Hsp31p, Hsp32p, and Sno4p; member of the DJ-1/ThiJ/PfpI superfamily, which includes human DJ-1 involved in Parkinson's disease and cancer; Belongs to the peptidase C56 family. HSP31-like subfamily.

Mitochondrial chaperone BCS1; Protein translocase and chaperone required for Complex III assembly; member of the AAA ATPase family; forms a homo-oligomeric complex in the mitochondrial inner membrane that translocates the C-terminal domain of Rip1p from the matrix across the inner membrane and delivers it to an assembly intermediate of respiratory Complex III; also required for assembly of the Qcr10p subunit; mutation is functionally complemented by human homolog BCS1L, linked to neonatal diseases.

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.

Probable glutathione-independent glyoxalase SNO4; Possible chaperone and cysteine protease; required for transcriptional reprogramming during the diauxic shift and for survival in stationary phase; similar to bacterial Hsp31 and yeast Hsp31p, Hsp32p, and Hsp33p; DJ-1/ThiJ/PfpI superfamily member; predicted involvement in pyridoxine metabolism; induced by mild heat stress and copper deprivation.

Disaggregase; heat shock protein that cooperates with Ydj1p (Hsp40) and Ssa1p (Hsp70) to refold and reactivate previously denatured, aggregated proteins; responsive to stresses including: heat, ethanol, and sodium arsenite; involved in [PSI+] propagation; protein becomes more abundant and forms cytoplasmic foci in response to DNA replication stress; potentiated Hsp104p variants decrease TDP-43 proteotoxicity by eliminating its cytoplasmic aggregation; Belongs to the ClpA/ClpB family.

5-aminolevulinate synthase, mitochondrial; 5-aminolevulinate synthase; catalyzes the first step in the heme biosynthetic pathway; an N-terminal signal sequence is required for localization to the mitochondrial matrix; expression is regulated by Hap2p-Hap3p; has a pyridoxal phosphate cofactor whose insertion is mediated by Mcx1p; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.