Sequence Description Alias PCC hrr 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] 0.6157065173415871 4 YGR232W Evolutionarily conserved 19S regulatory particle assembly-chaperone; proteasome-interacting protein involved in the assembly of the base subcomplex of the 19S proteasomal regulatory particle (RP); ortholog of human oncoprotein gankyrin, also known as p28, which interacts with the Rb tumor suppressor and CDK4/6 [Source:SGD;Acc:S000003464] 0.6001694159529305 3 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] 0.5923092971322078 80 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] 0.5868699221054449 23 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] 0.5819006252757385 29 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] 0.5814768908589236 52 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] 0.5803353071479553 7 YER178W E1 alpha subunit of the pyruvate dehydrogenase (PDH) complex; catalyzes the direct oxidative decarboxylation of pyruvate to acetyl-CoA; phosphorylated; regulated by glucose; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes [Source:SGD;Acc:S000000980] 0.5801795997217714 17 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] 0.5794293275729503 9 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] 0.5740428840843378 50 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] 0.5663568300163877 12 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] 0.5641725508072811 32 YDR286C Putative protein of unknown function; predicted to have thiol-disulfide oxidoreductase active site [Source:SGD;Acc:S000002694] 0.5544947782795968 14 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] 0.5523996999354154 65 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] 0.548704556829047 42 YDL137W ADP-ribosylation factor; GTPase of the Ras superfamily involved in regulation of coated formation vesicles in intracellular trafficking within the Golgi; ARF2 has a paralog, ARF1, that arose from the whole genome duplication; arf1 arf2 double null mutation is complemented by human ARF1, ARF4, ARF5, or ARF6 [Source:SGD;Acc:S000002296] 0.5448413894107008 27 YBR221C E1 beta subunit of the pyruvate dehydrogenase (PDH) complex; PDH is an evolutionarily conserved multi-protein complex found in mitochondria [Source:SGD;Acc:S000000425] 0.5416943558966725 40 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] 0.5400027731822924 74 YJL062W-A Mitochondrial protein required for cytochrome c oxidase assembly; also involved in translational regulation of Cox1p and prevention of Cox1p aggregation before assembly; located in the mitochondrial inner membrane [Source:SGD;Acc:S000007611] 0.5386549177179958 20 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] 0.5385753792959442 69 YKL085W Mitochondrial malate dehydrogenase; catalyzes interconversion of malate and oxaloacetate; involved in the tricarboxylic acid (TCA) cycle; phosphorylated [Source:SGD;Acc:S000001568] 0.5381181025572371 69 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] 0.5374039778716745 72 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] 0.5317031569789835 26 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] 0.5304388408557488 78 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] 0.5215648762604156 44 YDR115W Putative mitochondrial ribosomal protein of the large subunit; similar to E. coli L34 ribosomal protein; required for respiratory growth, as are most mitochondrial ribosomal proteins; protein increases in abundance and relocalizes to the plasma membrane upon DNA replication stress [Source:SGD;Acc:S000002522] 0.5149830421483347 57 YOR286W Protein with rhodanese activity; contains a rhodanese-like domain similar to Rdl1p, Uba4p, Tum1p, and Ych1p; overexpression causes a cell cycle delay; null mutant displays elevated frequency of mitochondrial genome loss [Source:SGD;Acc:S000005812] 0.5114422626906501 91 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] 0.5073499468840382 57 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] 0.5071922440393128 69 YMR035W Catalytic subunit of mitochondrial inner membrane peptidase complex; required for maturation of mitochondrial proteins of the intermembrane space; complex contains two catalytic subunits (Imp1p and Imp2p that differ in substrate specificity), and Som1p [Source:SGD;Acc:S000004638] 0.5044045848606044 78 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] 0.502980085115508 53 YER057C Member of the p14.5 protein family; functionally complements Mmf1p function when targeted to mitochondria; heat shock inducible; high-dosage growth inhibitor; forms a homotrimer in vitro; HMF1 has a paralog, MMF1, that arose from the whole genome duplication [Source:SGD;Acc:S000000859] 0.5011499353017138 42 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] 0.4975865585241911 80 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] 0.4964521334161389 87 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] 0.49040897929126753 51 YBR109C Calmodulin; Ca2+ binding protein that regulates Ca2+ independent processes (mitosis, bud growth, actin organization, endocytosis, etc.) and Ca2+ dependent processes (stress-activated pathways), targets include Nuf1p, Myo2p and calcineurin; binds to the Hog1p MAPK in response to hyperosmotic stress; potentiates membrane tubulation and constriction mediated by the Rvs161p-Rvs167p complex; human CALM1 or CALM2 functionally complement repression induced inviability [Source:SGD;Acc:S000000313] 0.490112601044177 52 YCL033C Methionine-R-sulfoxide reductase; involved in the response to oxidative stress; protects iron-sulfur clusters from oxidative inactivation along with MXR1; involved in the regulation of lifespan [Source:SGD;Acc:S000000538] 0.48844599360414603 87 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] 0.48752067508656693 79 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] 0.48706365864503387 55 YMR083W Mitochondrial alcohol dehydrogenase isozyme III; involved in the shuttling of mitochondrial NADH to the cytosol under anaerobic conditions and ethanol production [Source:SGD;Acc:S000004688] 0.4867249979682392 74 YPL218W ARF family GTPase; component of the COPII vesicle coat; required for transport vesicle formation during ER to Golgi protein transport; lowers membrane rigidity aiding vesicle formation; localizes to ER-mitochondrial contact sites where it enhances membrane curvature, thereby reducing contact size via its N-terminal amphipathic helix; regulates mitochondrial fission and fusion dynamics [Source:SGD;Acc:S000006139] 0.4775628236605367 84 YEL066W D-Amino acid N-acetyltransferase that detoxifies D-amino acids; catalyzes N-acetylation of D-amino acids through ordered bi-bi mechanism in which acetyl-CoA is first substrate bound and CoA is last product liberated; acetylates histones and polyamines, also autoacetylates [Source:SGD;Acc:S000000792] 0.4732959178038975 88 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] 0.46482051129967134 74 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] 0.4622577943148664 76 YJR104C 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 [Source:SGD;Acc:S000003865] 0.45360677121902576 79 YOR136W Subunit of mitochondrial NAD(+)-dependent isocitrate dehydrogenase; complex catalyzes the oxidation of isocitrate to alpha-ketoglutarate in the TCA cycle; phosphorylated [Source:SGD;Acc:S000005662] 0.45208123102032155 85 YDL125C Adenosine 5'-monophosphoramidase; interacts physically and genetically with Kin28p, a CDK and TFIIK subunit, and genetically with CAK1; member of histidine triad (HIT) superfamily of nucleotide-binding proteins; protein abundance increases in response to DNA replication stress; human homolog HINT1 can complement yeast hnt1 mutant [Source:SGD;Acc:S000002283] 0.45189872226692357 86 YML028W Thioredoxin peroxidase; acts as both ribosome-associated and free cytoplasmic antioxidant; self-associates to form high-molecular weight chaperone complex under oxidative stress; chaperone activity essential for growth in zinc deficiency; required for telomere length maintenance; binds and modulates Cdc19p activity; protein abundance increases, forms cytoplasmic foci during DNA replication stress; TSA1 has a paralog, TSA2, that arose from the whole genome duplication [Source:SGD;Acc:S000004490] 0.4468833491865103 90 YDR154C Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data; null mutant exhibits synthetic phenotype with alpha-synuclein [Source:SGD;Acc:S000002561] 0.4448028831702272 95 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] 0.4439564904517982 96 YGR282C Endo-beta-1,3-glucanase; major protein of the cell wall, involved in cell wall maintenance; involved in incorporation of newly synthesized mannoprotein molecules into the cell wall [Source:SGD;Acc:S000003514] 0.4413736066636849 99