Sequence Description Alias PCC hrr 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] 0.7776523089908461 3 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] 0.7620513064321232 8 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] 0.7511669163891094 5 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] 0.7476660998006541 6 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] 0.7262408034951002 22 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] 0.7158851088400098 18 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] 0.7057574154663447 21 YKL141W Subunit of succinate dehydrogenase and of TIM22 translocase; functions as cytochrome b subunit of succinate dehydrogenase, which couples oxidation of succinate to transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; also required for mitochondrial inner membrane protein import as part of the TIM22 complex; SDH3 has a paralog, SHH3, that arose from the whole genome duplication [Source:SGD;Acc:S000001624] 0.7054923093271563 16 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] 0.7013016081102781 24 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] 0.6846470236810209 10 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] 0.6841471851948728 27 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] 0.6815510144195864 38 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] 0.6775735202212596 25 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] 0.6724038703267713 25 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] 0.6645425268204651 30 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] 0.6553525974291303 16 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] 0.6428597134208613 26 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] 0.6302070124286141 60 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] 0.6222533280574681 57 YKL085W Mitochondrial malate dehydrogenase; catalyzes interconversion of malate and oxaloacetate; involved in the tricarboxylic acid (TCA) cycle; phosphorylated [Source:SGD;Acc:S000001568] 0.6172858745632251 38 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] 0.6096313927555457 47 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] 0.6011955317331649 30 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] 0.6001032356256539 41 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] 0.5950985901557337 90 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] 0.5910455809428319 67 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] 0.590179044191582 28 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] 0.5837799845591123 33 YOR187W 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 [Source:SGD;Acc:S000005713] 0.5825152710645966 89 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] 0.5819006252757385 29 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] 0.5804855837203922 62 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] 0.5788162243167738 71 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] 0.5763680132793616 77 YCL057C-A 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 [Source:SGD;Acc:S000007547] 0.5759618186221334 33 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] 0.5685182420184193 61 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] 0.5601828473841229 35 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] 0.5572567202021027 36 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] 0.533358890676511 40 YMR145C 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 [Source:SGD;Acc:S000004753] 0.5255079126302452 42 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] 0.5254249049601133 98 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] 0.5212292159968193 96 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] 0.5166456490859404 92 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] 0.5134279490321422 47 YAL046C Protein involved in Fe-S cluster transfer to mitochondrial clients; protects [4Fe-4S] clusters from damage due to oxidative stress by acting along with Nfu1p at a late step in the transfer of [4Fe-4S] clusters from the ISA complex to mitochondrial client proteins like lipoate synthase and succinate dehydrogenase; sequence similarity to human BOLA family member, BOLA3, mutations of which are associated with Multiple Mitochondria Dysfunctions Syndrome (MMDS2) [Source:SGD;Acc:S000000044] 0.5081962800115877 48 YNL037C Subunit of mitochondrial NAD(+)-dependent isocitrate dehydrogenase; complex catalyzes the oxidation of isocitrate to alpha-ketoglutarate in the TCA cycle [Source:SGD;Acc:S000004982] 0.48970408043107094 52 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] 0.4852502461339836 70 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] 0.4714415298786069 57 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] 0.46329215681421715 85 YMR203W Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; constitutes the core element of the protein conducting pore; pre-Tom40p is phosphorylated by PKA, which impairs its import into mitochondria under non-respiratory conditions [Source:SGD;Acc:S000004816] 0.4601807437189407 62 YGL040C Aminolevulinate dehydratase; a homo-octameric enzyme, catalyzes the conversion of 5-aminolevulinate to porphobilinogen, the second step in heme biosynthesis; enzymatic activity is zinc-dependent; localizes to the cytoplasm and nucleus; human homolog ALAD can complement yeast hem2 mutant [Source:SGD;Acc:S000003008] 0.4511147331913761 63 YDR232W 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 [Source:SGD;Acc:S000002640] 0.44742058733274853 64 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] 0.44393363149575676 65 YKR065C Constituent of the TIM23 complex; proposed alternatively to be a component of the import motor (PAM complex) or to interact with and modulate the core TIM23 (Translocase of the Inner mitochondrial Membrane) complex; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000001773] 0.44226318344006615 94 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] 0.43930831419175037 70 YOL126C Cytoplasmic malate dehydrogenase; one of three isozymes that catalyze interconversion of malate and oxaloacetate; involved in the glyoxylate cycle and gluconeogenesis during growth on two-carbon compounds; interacts with Pck1p and Fbp1 [Source:SGD;Acc:S000005486] 0.4380830091739938 80 YKL192C Mitochondrial matrix acyl carrier protein; involved in biosynthesis of octanoate, which is a precursor to lipoic acid; activated by phosphopantetheinylation catalyzed by Ppt2p [Source:SGD;Acc:S000001675] 0.4337608919839186 74 YJL143W Essential component of the TIM23 complex; with Tim23p, contributes to the architecture and function of the import channel; may link the import motor to the core Translocase of the Inner Mitochondrial membrane (TIM23 complex) [Source:SGD;Acc:S000003679] 0.4290380694199069 75 YAL060W NAD-dependent (R,R)-butanediol dehydrogenase; catalyzes oxidation of (R,R)-2,3-butanediol to (3R)-acetoin, oxidation of meso-butanediol to (3S)-acetoin, and reduction of acetoin; enhances use of 2,3-butanediol as an aerobic carbon source [Source:SGD;Acc:S000000056] 0.41965698591548495 82 YAL062W NADP(+)-dependent glutamate dehydrogenase; synthesizes glutamate from ammonia and alpha-ketoglutarate; rate of alpha-ketoglutarate utilization differs from Gdh1p; expression regulated by nitrogen and carbon sources; GDH3 has a paralog, GDH1, that arose from the whole genome duplication [Source:SGD;Acc:S000000058] 0.4093655021976709 91 YOR348C Proline permease; required for high-affinity transport of proline; also transports the toxic proline analog azetidine-2-carboxylate (AzC); PUT4 transcription is repressed in ammonia-grown cells [Source:SGD;Acc:S000005875] 0.4043705162545416 93