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MIC27 | 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; forms a subcomplex with Mic10p and Mic12p whose assembly and stability requires cardiolipin; Belongs to the apolipoprotein O/MICOS complex subunit Mic27 family. (234 aa) | ||||
COX2 | Subunit II of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits. (251 aa) | ||||
CYC3 | Cytochrome c heme lyase (holocytochrome c synthase); attaches heme to apo-cytochrome c (Cyc1p or Cyc7p) in mitochondrial intermembrane space; human homolog HCCS implicated in microphthalmia with linear skin defects (MLS), and can complement yeast null mutant. (269 aa) | ||||
PIM1 | 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. (1133 aa) | ||||
MGR1 | Subunit of the mitochondrial (mt) i-AAA protease supercomplex; i-AAA degrades misfolded mitochondrial proteins; forms a subcomplex with Mgr3p that binds to substrates to facilitate proteolysis; required for growth of cells lacking mtDNA. (417 aa) | ||||
MIC10 | Conserved 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; forms a subcomplex with Mic12p and Mic27p whose assembly and stability requires cardiolipin; homo-oligomers cause membrane bending; ortholog of human MINOS1. (97 aa) | ||||
TPI1 | Triose phosphate isomerase, abundant glycolytic enzyme; mRNA half-life is regulated by iron availability; transcription is controlled by activators Reb1p, Gcr1p, and Rap1p through binding sites in the 5' non-coding region; inhibition of Tpi1p activity by PEP (phosphoenolpyruvate) stimulates redox metabolism in respiring cells; E104D mutation in human homolog TPI1 causes a rare autosomal disease; human TPI1 can complement yeast null mutant. (248 aa) | ||||
HSP78 | 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. (811 aa) | ||||
CYC7 | Cytochrome c isoform 2, expressed under hypoxic conditions; also known as iso-2-cytochrome c; electron carrier of the mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; protein abundance increases in response to DNA replication stress; CYC7 has a paralog, CYC1, that arose from the whole genome duplication. (113 aa) | ||||
YND1 | Apyrase with wide substrate specificity; helps prevent inhibition of glycosylation by hydrolyzing nucleoside tri- and diphosphates that inhibit glycotransferases; partially redundant with Gda1p; mediates adenovirus E4orf4-induced toxicity. (630 aa) | ||||
MDJ1 | DnaJ homolog 1, mitochondrial; Co-chaperone that stimulates HSP70 protein Ssc1p ATPase activity; involved in protein folding/refolding in the mitochodrial matrix; required for proteolysis of misfolded proteins; member of the HSP40 (DnaJ) family of chaperones. (511 aa) | ||||
MIC19 | 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; Mic19p is peripheral to the inner membrane and may connect Mic60p with the Mic10p-Mic12p-Mic27p subcomplex; both yeast and human Mic19p become oxidized, and oxidation may regulate MICOS. (170 aa) | ||||
ERV1 | Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation. (189 aa) | ||||
PHB2 | 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). (310 aa) | ||||
TIM10 | Mitochondrial import inner membrane translocase subunit TIM10; Essential protein of the mitochondrial intermembrane space; forms a complex with Tim9p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane. (93 aa) | ||||
GUT2 | Mitochondrial glycerol-3-phosphate dehydrogenase; expression is repressed by both glucose and cAMP and derepressed by non-fermentable carbon sources in a Snf1p, Rsf1p, Hap2/3/4/5 complex dependent manner. (649 aa) | ||||
MPM1 | Mitochondrial intermembrane space protein of unknown function. (252 aa) | ||||
SSC1 | Heat shock protein SSC1, mitochondrial; Hsp70 family ATPase; constituent of the import motor component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); involved in protein translocation and folding; subunit of SceI endonuclease; SSC1 has a paralog, ECM10, that arose from the whole genome duplication. (654 aa) | ||||
CYC1 | Cytochrome c, isoform 1; also known as iso-1-cytochrome c; electron carrier of mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; CYC1 has a paralog, CYC7, that arose from the whole genome duplication; human homolog CYC1 can complement yeast null mutant; mutations in human CYC1 cause insulin-responsive hyperglycemia. (109 aa) | ||||
SOD1 | Cytosolic copper-zinc superoxide dismutase; detoxifies superoxide; stabilizes Yck1p and Yck2p kinases in glucose to repress respiration; phosphorylated by Dun1p, enters nucleus under oxidative stress to promote transcription of stress response genes; human ortholog SOD1 implicated in ALS complements a null allele; abundance increases under DNA replication stress and during exposure to boric acid; localization to mitochondrial intermembrane space is modulated by MICOS complex; Belongs to the Cu-Zn superoxide dismutase family. (154 aa) | ||||
CYT2 | Cytochrome c1 heme lyase; involved in maturation of cytochrome c1, which is a subunit of the mitochondrial ubiquinol-cytochrome-c reductase; links heme covalently to apocytochrome c1; human homolog HCCS can complement yeast cyt2 null mutant. (224 aa) | ||||
MIA40 | Import and assembly protein in mitochondrial intermembrane space; component of MIA pathway which mediates import and oxidative folding of substrates including small proteins containing twin cysteine motifs; acts in concert with Erv1p, which oxidizes the cysteine residues of Mia40p to comprise a disulfide relay system that catalyzes import; also mediates folding of Atp23p via a chaperone-like activity; forms a dimer that binds iron-sulfur cluster in vitro. (403 aa) | ||||
CCP1 | Mitochondrial cytochrome-c peroxidase; degrades reactive oxygen species in mitochondria, involved in the response to oxidative stress. (361 aa) | ||||
UPS2 | Mitochondrial intermembrane space protein; involved in phospholipid metabolism; forms complex with Mdm35p that transfers phosphatidylserine from outer membrane to inner membrane for phosphatidylethanolamine synthesis; null mutant has defects in mitochondrial morphology; similar to Ups1p, Ups3p and to human PRELI; UPS2 has a paralog, UPS3, that arose from the whole genome duplication; Belongs to the slowmo family. (230 aa) | ||||
HSP60 | Heat shock protein 60, mitochondrial; Tetradecameric mitochondrial chaperonin; required for ATP-dependent folding of precursor polypeptides and complex assembly; prevents aggregation and mediates protein refolding after heat shock; role in mtDNA transmission; phosphorylated. (572 aa) | ||||
MGR3 | 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. (501 aa) | ||||
NDE1 | Mitochondrial external NADH dehydrogenase; type II NAD(P)H:quinone oxidoreductase that catalyzes the oxidation of cytosolic NADH; Nde1p and Nde2p provide cytosolic NADH to the mitochondrial respiratory chain; NDE1 has a paralog, NDE2, that arose from the whole genome duplication. (560 aa) | ||||
HSC82 | ATP-dependent molecular chaperone HSC82; Cytoplasmic chaperone of the Hsp90 family; plays a role in determining prion variants; redundant in function and nearly identical with Hsp82p, and together they are essential; expressed constitutively at 10-fold higher basal levels than HSP82 and induced 2-3 fold by heat shock; contains two acid-rich unstructured regions that promote the solubility of chaperone-substrate complexes; HSC82 has a paralog, HSP82, that arose from the whole genome duplication. (705 aa) | ||||
TOM70 | Mitochondrial import receptor subunit TOM70; Component of the TOM (translocase of outer membrane) complex; involved in the recognition and initial import steps for all mitochondrially directed proteins; acts as a receptor for incoming precursor proteins; TOM70 has a paralog, TOM71, that arose from the whole genome duplication. (617 aa) | ||||
TOM22 | Mitochondrial import receptor subunit TOM22; Component of the TOM (Translocase of Outer Membrane) complex; responsible for initial import of mitochondrially directed proteins; mediates interaction between TOM and TIM complexes and acts as a receptor for precursor proteins; Belongs to the Tom22 family. (152 aa) | ||||
ZIM17 | Protein co-chaperone with a zinc finger motif; essential for protein import into mitochondria; may act with Pam18p to facilitate recognition and folding of imported proteins by Ssc1p (mtHSP70) in the mitochondrial matrix; required for the maintenance of Ssc1p solubility and assists in the functional interaction of Ssc1p with substrate proteins. (174 aa) | ||||
HSP10 | 10 kDa heat shock protein, mitochondrial; Mitochondrial matrix co-chaperonin; inhibits the ATPase activity of Hsp60p, a mitochondrial chaperonin; involved in protein folding and sorting in the mitochondria; 10 kD heat shock protein with similarity to E. coli groES. (106 aa) | ||||
MGE1 | GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family. (228 aa) | ||||
DFR1 | Dihydrofolate reductase involved in tetrahydrofolate biosynthesis; required for respiratory metabolism; mutation is functionally complemented by human DHFR. (211 aa) | ||||
TIM50 | 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. (476 aa) | ||||
HSP82 | ATP-dependent molecular chaperone HSP82; Hsp90 chaperone; redundant in function with Hsc82p; required for pheromone signaling, negative regulation of Hsf1p; docks with Tom70p for mitochondrial preprotein delivery; promotes telomerase DNA binding, nucleotide addition; protein abundance increases in response to DNA replication stress; contains two acid-rich unstructured regions that promote solubility of chaperone-substrate complexes; HSP82 has a paralog, HSC82, that arose from the whole genome duplication. (709 aa) | ||||
YME1 | 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. (747 aa) |