Sequence Description Alias PCC hrr YDR178W Membrane anchor subunit of succinate dehydrogenase (SDH); involved in coupling the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; has similarity to human SDH subunit D (SDHD), which is implicated in paraganglioma [Source:SGD;Acc:S000002585] 0.8310329096978922 1 YLL041C Iron-sulfur protein subunit of succinate dehydrogenase; the complex couples the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; other members are Sdh1p, Sdh3p, and Sdh4p [Source:SGD;Acc:S000003964] 0.8008034348220574 6 YPL134C Mitochondrial inner membrane transporter; 2-oxodicarboxylate transporter, exports 2-oxoadipate and 2-oxoglutarate from the mitochondrial matrix to the cytosol for lysine and glutamate biosynthesis and lysine catabolism; suppresses, in multicopy, an fmc1 null mutation; ODC1 has a paralog, ODC2, that arose from the whole genome duplication [Source:SGD;Acc:S000006055] 0.7847387704510514 8 YFL030W Alanine:glyoxylate aminotransferase (AGT); catalyzes the synthesis of glycine from glyoxylate, which is one of three pathways for glycine biosynthesis in yeast; similar to mammalian and plant alanine:glyoxylate aminotransferases; human homolog AGXT complements yeast null mutant [Source:SGD;Acc:S000001864] 0.7827663587088233 4 YGL187C Subunit IV of cytochrome c oxidase; the terminal member of the mitochondrial inner membrane electron transport chain; precursor N-terminal 25 residues are cleaved during mitochondrial import; phosphorylated; spermidine enhances translation [Source:SGD;Acc:S000003155] 0.7800932752759322 13 YKL016C Subunit d of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis [Source:SGD;Acc:S000001499] 0.7562089306085812 18 YPL078C Subunit b of the stator stalk of mitochondrial F1F0 ATP synthase; ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; contributes to the oligomerization of the complex, which in turn determines the shape of inner membrane cristae; phosphorylated [Source:SGD;Acc:S000005999] 0.7535654887014335 15 YKL148C Flavoprotein subunit of succinate dehydrogenase; couples the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; FAD binding to Sdh1p is required for the assembly of the succinate dehydrogenase subunits; mutations in human ortholog SDHA are associated with Leigh syndrome [Source:SGD;Acc:S000001631] 0.7520310546593229 14 YHR051W Subunit VI of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; expression is regulated by oxygen levels [Source:SGD;Acc:S000001093] 0.7500922738033365 11 YDR148C Dihydrolipoyl transsuccinylase; component of the mitochondrial alpha-ketoglutarate dehydrogenase complex, which catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA in the TCA cycle; phosphorylated [Source:SGD;Acc:S000002555] 0.7363203870957826 22 YDR298C Subunit 5 of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; homologous to bovine subunit OSCP (oligomycin sensitivity-conferring protein); phosphorylated [Source:SGD;Acc:S000002706] 0.7356642474586121 14 YEL024W Ubiquinol-cytochrome-c reductase; a Rieske iron-sulfur protein of the mitochondrial cytochrome bc1 complex; transfers electrons from ubiquinol to cytochrome c1 during respiration; during import, Rip1p is first imported into the mitochondrial matrix where it is processed, acquires its Fe-S cluster, and is folded, then is translocated into the inner membrane by the action of a homo-oligomer of Bcs1p, and finally is delivered by Bcs1p to Complex III for assembly [Source:SGD;Acc:S000000750] 0.7270122880786367 13 YNR001C Citrate synthase; catalyzes the condensation of acetyl coenzyme A and oxaloacetate to form citrate; the rate-limiting enzyme of the TCA cycle; nuclear encoded mitochondrial protein; CIT1 has a paralog, CIT2, that arose from the whole genome duplication [Source:SGD;Acc:S000005284] 0.724844626278733 18 YPR020W Subunit g of the mitochondrial F1F0 ATP synthase; reversibly phosphorylated on two residues; unphosphorylated form is required for dimerization of the ATP synthase complex, which in turn determines oligomerization of the complex and the shape of inner membrane cristae [Source:SGD;Acc:S000006224] 0.7181192539080352 14 YDL004W Delta subunit of the central stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated [Source:SGD;Acc:S000002162] 0.7149370317251521 19 YJR077C Mitochondrial phosphate carrier; imports inorganic phosphate into mitochondria; functionally redundant with Pic2p but more abundant than Pic2p under normal conditions; phosphorylated [Source:SGD;Acc:S000003838] 0.7054923093271563 16 YLR038C Subunit VIb of cytochrome c oxidase; cytochrome c oxidase is also known as respiratory Complex IV and is the terminal member of the mitochondrial inner membrane electron transport chain; required for assembly of cytochrome c oxidase but not required for activity after assembly; phosphorylated; easily released from the intermembrane space, suggesting a loose association with Complex IV [Source:SGD;Acc:S000004028] 0.7051277367732598 17 YLR295C Subunit h of the F0 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000004286] 0.6979114242557775 28 YJR121W Beta subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated [Source:SGD;Acc:S000003882] 0.6811801743290831 25 YGL191W Subunit VIa of cytochrome c oxidase; present in a subclass of cytochrome c oxidase complexes that may have a role in mimimizing generation of reactive oxygen species; not essential for cytochrome c oxidase activity but may modulate activity in response to ATP; required for assembly of Rcf2p into cytochrome c oxidase - cytochrome bc1 supercomplexes [Source:SGD;Acc:S000003159] 0.6768871681108504 20 YLR174W Cytosolic NADP-specific isocitrate dehydrogenase; catalyzes oxidation of isocitrate to alpha-ketoglutarate; levels are elevated during growth on non-fermentable carbon sources and reduced during growth on glucose; IDP2 has a paralog, IDP3, that arose from the whole genome duplication [Source:SGD;Acc:S000004164] 0.6731235039576234 21 YPL262W Fumarase; converts fumaric acid to L-malic acid in the TCA cycle; cytosolic and mitochondrial distribution determined by the N-terminal targeting sequence, protein conformation, and status of glyoxylate shunt; phosphorylated in mitochondria [Source:SGD;Acc:S000006183] 0.6710702906378766 26 YBL045C Core subunit of the ubiquinol-cytochrome c reductase complex; the ubiquinol-cytochrome c reductase complex (bc1 complex) is a component of the mitochondrial inner membrane electron transport chain [Source:SGD;Acc:S000000141] 0.6609365743112182 55 YBL015W Protein with CoA transferase activity; particularly for CoASH transfer from succinyl-CoA to acetate; has minor acetyl-CoA-hydrolase activity; phosphorylated; required for acetate utilization and for diploid pseudohyphal growth [Source:SGD;Acc:S000000111] 0.6607155124144867 24 YPR191W Subunit 2 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; phosphorylated; transcription is regulated by Hap1p, Hap2p/Hap3p, and heme [Source:SGD;Acc:S000006395] 0.6585263319886139 46 YDR377W Subunit f of the F0 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis [Source:SGD;Acc:S000002785] 0.6563963927723546 26 YDR529C Subunit 7 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; oriented facing the mitochondrial matrix; N-terminus appears to play a role in complex assembly [Source:SGD;Acc:S000002937] 0.6550905058569081 35 YNL052W Subunit Va of cytochrome c oxidase; cytochrome c oxidase is the terminal member of the mitochondrial inner membrane electron transport chain; Cox5Ap is predominantly expressed during aerobic growth while its isoform Vb (Cox5Bp) is expressed during anaerobic growth; COX5A has a paralog, COX5B, that arose from the whole genome duplication [Source:SGD;Acc:S000004997] 0.6514358844970262 32 YBR039W Gamma subunit of the F1 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis [Source:SGD;Acc:S000000243] 0.6506878447510638 29 YLR377C Fructose-1,6-bisphosphatase; key regulatory enzyme in the gluconeogenesis pathway, required for glucose metabolism; undergoes either proteasome-mediated or autophagy-mediated degradation depending on growth conditions; glucose starvation results in redistribution to the periplasm; interacts with Vid30p [Source:SGD;Acc:S000004369] 0.6422445997915703 30 YDL181W Protein that inhibits ATP hydrolysis by the F1F0-ATP synthase; inhibitory function is enhanced by stabilizing proteins Stf1p and Stf2p; has a calmodulin-binding motif and binds calmodulin in vitro; INH1 has a paralog, STF1, that arose from the whole genome duplication [Source:SGD;Acc:S000002340] 0.6397651864428248 31 YML120C NADH:ubiquinone oxidoreductase; transfers electrons from NADH to ubiquinone in respiratory chain but does not pump protons, in contrast to higher eukaryotic multisubunit respiratory complex I; upon apoptotic stress, is activated in mitochondria by N-terminal cleavage, then translocates to cytoplasm to induce apoptosis; homolog of human AIFM2; yeast NDI1 complements several phenotypes of human cell line with mutated MT-ND4, implicated in Leber hereditary optic neuropathy [Source:SGD;Acc:S000004589] 0.6360443878594844 72 YKL067W Nucleoside diphosphate kinase; catalyzes the transfer of gamma phosphates from nucleoside triphosphates, usually ATP, to nucleoside diphosphates by a mechanism that involves formation of an autophosphorylated enzyme intermediate; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000001550] 0.6306354933493733 33 YKL085W Mitochondrial malate dehydrogenase; catalyzes interconversion of malate and oxaloacetate; involved in the tricarboxylic acid (TCA) cycle; phosphorylated [Source:SGD;Acc:S000001568] 0.6246214386319172 34 YBL099W Alpha subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated; N-terminally propionylated in vivo [Source:SGD;Acc:S000000195] 0.6217712611539784 35 YKL150W Mitochondrial NADH-cytochrome b5 reductase; involved in ergosterol biosynthesis [Source:SGD;Acc:S000001633] 0.619793045302628 63 YLR395C Subunit VIII of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain [Source:SGD;Acc:S000004387] 0.6159697347581271 37 YNL100W 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 [Source:SGD;Acc:S000005044] 0.612624232635681 96 YFL018C Dihydrolipoamide dehydrogenase; the lipoamide dehydrogenase component (E3) of the pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase multi-enzyme complexes; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes; LPD1 has a paralog, IRC15, that arose from the whole genome duplication [Source:SGD;Acc:S000001876] 0.5956213544599053 44 YIL125W Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase complex; catalyzes a key step in the tricarboxylic acid (TCA) cycle, the oxidative decarboxylation of alpha-ketoglutarate to form succinyl-CoA [Source:SGD;Acc:S000001387] 0.594416079467555 55 YJR085C Protein of unknown function; GFP-fusion protein is induced in response to the DNA-damaging agent MMS; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000003845] 0.5916647043219542 42 YNL055C Mitochondrial porin (voltage-dependent anion channel); outer membrane protein required for maintenance of mitochondrial osmotic stability and mitochondrial membrane permeability; couples the glutathione pools of the intermembrane space (IMS) and the cytosol; interacts with Om45 and Om14 in the outer membrane; phosphorylated; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000005000] 0.5908672993412452 43 YOR065W Cytochrome c1; component of the mitochondrial respiratory chain; expression is regulated by the heme-activated, glucose-repressed Hap2p/3p/4p/5p CCAAT-binding complex [Source:SGD;Acc:S000005591] 0.5902040040925522 50 YPR010C-A Putative protein of unknown function; SWAT-GFP, seamless-GFP and mCherry fusion proteins localize to the cytosol; conserved among Saccharomyces sensu stricto species [Source:SGD;Acc:S000122558] 0.5880153574557082 45 YHL032C Glycerol kinase; converts glycerol to glycerol-3-phosphate; glucose repression of expression is mediated by Adr1p and Ino2p-Ino4p; derepression of expression on non-fermentable carbon sources is mediated by Opi1p and Rsf1p [Source:SGD;Acc:S000001024] 0.5868365030255724 46 YHR008C Mitochondrial manganese superoxide dismutase; protects cells against oxygen toxicity and oxidative stress; human mitochondrial SOD2 can complement a yeast null mutant and human cytoplasmic SOD1 can also complement when targeted to the mitochondrial matrix [Source:SGD;Acc:S000001050] 0.5765643497241904 47 YDL130W-A Protein involved in regulation of the mitochondrial F1F0-ATP synthase; Stf1p and Stf2p act as stabilizing factors that enhance inhibitory action of the Inh1p protein; protein abundance increases in response to DNA replication stress; STF1 has a paralog, INH1, that arose from the whole genome duplication [Source:SGD;Acc:S000007232] 0.5750888892528426 48 YFR049W Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase; recruits E3 subunit (Lpd1p) to the E1-E2 (Kgd1p, Kgd2p) core; has similarity to human mitochondrial ribosomal protein MRP-S36 [Source:SGD;Acc:S000001945] 0.5725062466390396 53 YOL147C Peroxisomal protein required for medium-chain fatty acid oxidation; also required for peroxisome proliferation, possibly by inducing membrane curvature; localization regulated by phosphorylation; transcription regulated by Adr1p and Pip2p-Oaf1p [Source:SGD;Acc:S000005507] 0.5677750199432494 51 YLR304C Aconitase; required for the tricarboxylic acid (TCA) cycle and also independently required for mitochondrial genome maintenance; component of the mitochondrial nucleoid; mutation leads to glutamate auxotrophy; human homolog ACO2 can complement yeast null mutant [Source:SGD;Acc:S000004295] 0.5662250691396709 52 YBL030C Major ADP/ATP carrier of the mitochondrial inner membrane; exchanges cytosolic ADP for mitochondrially synthesized ATP; also imports heme and ATP; required for viability in many lab strains that carry a sal1 mutation; PET9 has a paralog, AAC3, that arose from the whole genome duplication; human homolog SLC25A4 implicated in progressive external ophthalmoplegia can complement yeast null mutant [Source:SGD;Acc:S000000126] 0.5657339654702693 53 YKR049C Putative redox protein containing a thioredoxin fold; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000001757] 0.5615638379385893 88 YDR322C-A Subunit e of mitochondrial F1F0-ATPase; ATPase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; essential for the dimeric and oligomeric state of ATP synthase, which in turn determines the shape of inner membrane cristae [Source:SGD;Acc:S000007255] 0.559236610618981 57 YPL271W Epsilon subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated [Source:SGD;Acc:S000006192] 0.5549243207683381 58 YNR002C Putative transmembrane protein involved in export of ammonia; ammonia is a starvation signal that promotes cell death in aging colonies; phosphorylated in mitochondria; member of the TC 9.B.33 YaaH family; homolog of Y. lipolytica Gpr1p; ATO2 has a paralog, ADY2, that arose from the whole genome duplication [Source:SGD;Acc:S000005285] 0.5450128214793398 61 YHR001W-A Subunit of the ubiqunol-cytochrome c oxidoreductase complex; this complex comprises part of the mitochondrial respiratory chain; members include Cobp, Rip1p, Cyt1p, Cor1p, Qcr2p, Qcr6p, Qcr7p, Qcr8p, Qcr9p, and Qcr10p and comprises part of the mitochondrial respiratory chain [Source:SGD;Acc:S000003529] 0.5317237850289006 65 YNL117W Malate synthase, enzyme of the glyoxylate cycle; involved in utilization of non-fermentable carbon sources; expression is subject to carbon catabolite repression; localizes in peroxisomes during growth on oleic acid, otherwise cytosolic; can accept butyryl-CoA as acyl-CoA donor in addition to traditional substrate acetyl-CoA [Source:SGD;Acc:S000005061] 0.5242615588167218 68 YJR095W Mitochondrial succinate-fumarate transporter; transports succinate into and fumarate out of the mitochondrion; required for ethanol and acetate utilization [Source:SGD;Acc:S000003856] 0.5217406515679704 69 YJR019C Peroxisomal acyl-CoA thioesterase; likely to be involved in fatty acid oxidation rather than fatty acid synthesis; conserved protein also found in human peroxisomes; TES1 mRNA levels increase during growth on fatty acids [Source:SGD;Acc:S000003780] 0.5176295424021874 75 YNL015W Cytosolic inhibitor of vacuolar proteinase B (PRB1); required for efficient vacuole inheritance; with thioredoxin forms protein complex LMA1, which assists in priming SNARE molecules and promotes vacuole fusion; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000004960] 0.516768926492068 73 YLR294C Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data; partially overlaps the verified gene ATP14 [Source:SGD;Acc:S000004285] 0.5161578015076025 74 YBR222C Oxalyl-CoA synthetase; capable of catalyzing conversion of oxalate to oxalyl-CoA; catalyzes first step in pathway of oxalate degradation that functions to protect yeast from inhibitory effects of oxalate; peroxisomal protein that binds mRNA; localizes to both peroxisomal peripheral membrane and matrix, expression is highly inducible by oleic acid; similar to E. coli long chain acyl-CoA synthetase [Source:SGD;Acc:S000000426] 0.5091116981880627 78 YBR050C Regulatory subunit of the Glc7p type-1 protein phosphatase; involved with Reg1p, Glc7p, and Snf1p in regulation of glucose-repressible genes, also involved in glucose-induced proteolysis of maltose permease; REG2 has a paralog, REG1, that arose from the whole genome duplication [Source:SGD;Acc:S000000254] 0.5007993769475467 84 YBL043W Non-essential protein of unknown function; induced by treatment with 8-methoxypsoralen and UVA irradiation; ECM13 has a paralog, YJR115W, that arose from the whole genome duplication [Source:SGD;Acc:S000000139] 0.5001020653798962 85 YML078W Mitochondrial peptidyl-prolyl cis-trans isomerase (cyclophilin); catalyzes the cis-trans isomerization of peptide bonds N-terminal to proline residues; involved in protein refolding after import into mitochondria [Source:SGD;Acc:S000004543] 0.4964521334161389 87 YGL146C Putative protein of unknown function; non-essential gene identified in a screen for mutants with increased levels of rDNA transcription; contains two putative transmembrane spans, but no significant homology to other known proteins [Source:SGD;Acc:S000003114] 0.4942811150813077 88 YGR183C Subunit 9 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; required for electron transfer at the ubiquinol oxidase site of the complex [Source:SGD;Acc:S000003415] 0.49316243498866963 91 YNL203C Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data [Source:SGD;Acc:S000005147] 0.4902403899518947 92 YJR048W 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 [Source:SGD;Acc:S000003809] 0.488539912587439 95 YKR097W Phosphoenolpyruvate carboxykinase; key enzyme in gluconeogenesis, catalyzes early reaction in carbohydrate biosynthesis, glucose represses transcription and accelerates mRNA degradation, regulated by Mcm1p and Cat8p, located in the cytosol [Source:SGD;Acc:S000001805] 0.48436125351341197 98 YDR031W Mitochondrial intermembrane space protein of unknown function; required for normal oxygen consumption; contains twin cysteine-x9-cysteine motifs; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000002438] 0.4816967410978502 99