Sequence Description Alias PCC hrr YML030W Cytochrome c oxidase subunit; required for assembly of the Complex III-Complex IV supercomplex, and for assembly of Cox13p and Rcf2p into cytochrome c oxidase; similar to Rcf2p, and either Rcf1p or Rcf2p is required for late-stage assembly of the Cox12p and Cox13p subunits and for cytochrome c oxidase activity; required for growth under hypoxic conditions; member of the hypoxia induced gene family; C. elegans and human orthologs are functional in yeast [Source:SGD;Acc:S000004492] 0.8115380270373368 2 YIR001C Cytoplasmic RNA-binding protein; contains an RNA recognition motif (RRM); may have a role in mRNA translation, as suggested by genetic interactions with genes encoding proteins involved in translational initiation [Source:SGD;Acc:S000001440] 0.8066407108540501 2 YGR174C Mitochondrial protein required for assembly of cytochrome bc1 complex; interacts with the Cbp3p-Cbp6p complex and newly synthesized cytochrome b (Cobp) to promote assembly of Cobp into the cytochrome bc1 complex [Source:SGD;Acc:S000003406] 0.793617284595052 3 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] 0.7806638544709517 6 YCR071C Mitochondrial ribosomal protein of the large subunit; conserved in metazoa, with similarity to human mitochondrial ribosomal protein MRPL49 [Source:SGD;Acc:S000000667] 0.7653279533432897 25 YGR132C 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) [Source:SGD;Acc:S000003364] 0.7625949406265968 6 YBL021C Subunit of the Hap2p/3p/4p/5p CCAAT-binding complex; complex is heme-activated and glucose-repressed; complex is a transcriptional activator and global regulator of respiratory gene expression; contains sequences contributing to both complex assembly and DNA binding [Source:SGD;Acc:S000000117] 0.7624679992167905 7 YOL053W Protein of unknown function; null mutant displays elevated frequency of mitochondrial genome loss [Source:SGD;Acc:S000005414] 0.7570326681127683 8 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] 0.7561880283956718 9 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] 0.7509548179239062 14 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] 0.7509290201652911 21 YGR235C 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; Mic26p is a non-essential component of the complex [Source:SGD;Acc:S000003467] 0.7478070461371533 12 YBR262C 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 Mic27p whose assembly and stability requires cardiolipin [Source:SGD;Acc:S000000466] 0.7447172678072885 13 YNL284C Mitochondrial ribosomal protein of the large subunit; appears as two protein spots (YmL10 and YmL18) on two-dimensional SDS gels [Source:SGD;Acc:S000005228] 0.7365669491080323 50 YJR080C Protein with a role in determining mitochondrial architecture; inner membrane protein that interacts physically and genetically with the MICOS complex and is required for its integrity [Source:SGD;Acc:S000003841] 0.7355112903631378 15 YBR258C Subunit of the COMPASS (Set1C) complex; COMPASS methylates histone H3 on lysine 4 and is required in transcriptional silencing near telomeres [Source:SGD;Acc:S000000462] 0.7349678375723001 16 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] 0.7314473701325597 17 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] 0.7301857967990415 27 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] 0.7270096735116125 19 YLR239C Lipoyl ligase; involved in the modification of mitochondrial enzymes by the attachment of lipoic acid groups [Source:SGD;Acc:S000004229] 0.7201120659508381 48 YDR363W-A 19S proteasome regulatory particle lid subcomplex component; role in Ub-dependent proteolysis and proteasome stability; involved in TREX-2 mediated mRNA export, and in the prevention of transcription-associated genome instability; ubiquitinated by Nedd4-like E3-ligase, Rsp5p; human ortholog DSS1, a BRCA1 binding protein implicated in cancer, complements the yeast null; drives trinucleotide repeat expansion; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000007235] 0.7163264074983346 21 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] 0.7159258828983477 24 YER050C Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S18 ribosomal protein [Source:SGD;Acc:S000000852] 0.7138176445120088 23 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] 0.7065801657550548 28 YDR075W Catalytic subunit of protein phosphatase PP4 complex; Pph3p and Psy2p form active complex, Psy4p may provide substrate specificity; regulates recovery from the DNA damage checkpoint, the gene conversion- and single-strand annealing-mediated pathways of meiotic double-strand break repair and efficient Non-Homologous End-Joining (NHEJ) pathway; involved in activation of Gln3p to alleviate nitrogen catabolite repression; Pph3p and Psy2p localize to foci on meiotic chromosomes [Source:SGD;Acc:S000002482] 0.7025173107719922 25 YOR215C Protein of unknown function; the authentic protein is detected in highly purified mitochondria in high-throughput studies; null mutant displays reduced frequency of mitochondrial genome loss [Source:SGD;Acc:S000005741] 0.7021413511535067 26 YDR494W Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation [Source:SGD;Acc:S000002902] 0.7008832966699867 89 YPL118W Mitochondrial ribosomal protein of the small subunit; MRP51 exhibits genetic interactions with mutations in the COX2 and COX3 mRNA 5'-untranslated leader sequences [Source:SGD;Acc:S000006039] 0.6987522501911263 86 YEL050C Mitochondrial ribosomal protein of the large subunit (L2); has similarity to E. coli L2 ribosomal protein; mutant allele (fat21) causes inability to utilize oleate, and induce oleic acid oxidation; may interfere with activity of the Adr1p transcription factor [Source:SGD;Acc:S000000776] 0.6942111519670993 68 YIL014C-A Putative protein of unknown function [Source:SGD;Acc:S000003536] 0.693988106458663 30 YJL024C Small subunit of the clathrin-associated adaptor complex AP-3; involved in vacuolar protein sorting; related to the sigma subunit of the mammalian clathrin AP-3 complex; suppressor of loss of casein kinase 1 function; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000003561] 0.6932238472621209 37 YOR064C Subunit of the NuA3 histone acetyltransferase complex; this complex acetylates histone H3; contains PHD finger domain that interacts with methylated histone H3; shares significant sequence identity with the human candidate tumor suppressor p33-ING1 in C-terminal region [Source:SGD;Acc:S000005590] 0.6929342675132331 32 YGR222W Mitochondrial inner membrane protein; binds to the 5' UTR of the COX3 mRNA to activate its translation together with Pet122p and Pet494p; also binds to the COX1 Group I intron AI5 beta to facilitate exon ligation during splicing [Source:SGD;Acc:S000003454] 0.6892162651858958 33 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] 0.6874726959298264 34 YER170W Mitochondrial adenylate kinase; catalyzes the reversible synthesis of GTP and AMP from GDP and ADP; may serve as a back-up for synthesizing GTP or ADP depending on metabolic conditions; 3' sequence of ADK2 varies with strain background [Source:SGD;Acc:S000000972] 0.6866459724236241 35 YOR037W Mitochondrial peripheral inner membrane protein; contains a FAD cofactor in a domain exposed in the intermembrane space; exhibits redox activity in vitro; likely participates in ligation of heme to acytochromes c and c1 (Cyc1p and Cyt1p) [Source:SGD;Acc:S000005563] 0.6847636251681415 53 YJL063C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003599] 0.6803200573889256 58 YPR100W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006304] 0.6775673171957981 46 YPL173W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006094] 0.6768994943209825 87 YJL102W Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000003638] 0.6741090585562619 41 YGR231C 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) [Source:SGD;Acc:S000003463] 0.6720616695310179 72 YNR020C Putative metalloprotease of the mitochondrial inner membrane; required for processing of Atp6p; has an additional role in assembly of the F0 sector of the F1F0 ATP synthase complex; substrate of the Mia40p-Erv1p disulfide relay system, and folding is assisted by Mia40p [Source:SGD;Acc:S000005303] 0.6712930256764644 43 YGR101W Mitochondrial serine protease; required for the processing of various mitochondrial proteins and maintenance of mitochondrial DNA and morphology; belongs to the rhomboid-GlpG superfamily of intramembrane peptidases [Source:SGD;Acc:S000003333] 0.6688071717290027 44 YPL098C Subunit of the TIM23 translocase complex; acts to couple Tim21p with the core Tim23 translocase; provides quality control for insertion of membrane proteins by regulating their release into the inner membrane by the TIM23 complex; absolutely required for mitochondrial import of presequence-containing proteins at elevated temperature; required for viability of cells lacking the mitochondrial genome (petite-negative phenotype) [Source:SGD;Acc:S000006019] 0.6641459846132515 45 YDL135C Rho GDP dissociation inhibitor; involved in the localization and regulation of Cdc42p and Rho1p; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000002294] 0.6637335908247283 46 YKR006C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001714] 0.6626705043380836 95 YLR312W-A Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004304] 0.6614670048341595 84 YPL215W Mitochondrial protein required for assembly of cytochrome bc1 complex; forms a complex with Cbp6p that binds to mt ribosomes near the polypeptide tunnel exit and promotes efficient translation of the COB mRNA; Cbp3p-Cbp6p complex also interacts with newly synthesized cytochrome b (Cobp) and Cbp4p to promote assembly of Cobp into the cytochrome bc1 complex; Cbp3p-Cbp6p complex is sequestered if assembly of Complex III is blocked, downregulating COB mRNA translation [Source:SGD;Acc:S000006136] 0.658012191585993 99 YOL071W Protein required for flavinylation of Sdh1p; binds to Sdh1p and promotes FAD cofactor attachment, which is necessary for succinate dehydrogenase (SDH) complex assembly and activity; mutations in human ortholog PGL2 are associated with neuroendocrine tumors (paraganglioma) [Source:SGD;Acc:S000005432] 0.6572066372132145 66 YDL107W Peripherally bound inner membrane protein of the mitochondrial matrix; involved in membrane insertion of C-terminus of Cox2p, interacts genetically and physically with Cox18p [Source:SGD;Acc:S000002265] 0.6549738037448101 92 YHR071C-A Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data; partially overlaps the verified ORF ERG7/YHR072W [Source:SGD;Acc:S000028781] 0.6535024816742774 53 YCR003W Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000000596] 0.6530331580485147 71 YLR390W Protein of unknown function; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000004382] 0.6528583675866763 55 YHR121W Protein of unknown function that may function in RNA processing; interacts with Pbp1p and Pbp4p and associates with ribosomes; contains an RNA-binding LSM domain and an AD domain; GFP-fusion protein is induced by the DNA-damaging agent MMS; relative distribution to the nucleus increases upon DNA replication stress [Source:SGD;Acc:S000001163] 0.6521996002365484 56 YER058W Protein required for assembly of cytochrome c oxidase [Source:SGD;Acc:S000000860] 0.6498687425676691 57 YOR196C Protein involved in biosynthesis of the coenzyme lipoic acid; has similarity to E. coli lipoic acid synthase [Source:SGD;Acc:S000005722] 0.646704957734219 59 YPL099C F1F0 ATPase synthase peripheral stalk assembly factor; subunit of the matrix-exposed inner mitochondrial membrane localized INA complex (Ina22p-Ina17p) involved in assembly of the F1F0 peripheral stalk; co-purifies with Ina22p and ATP synthase subunits; null mutant displays elevated frequency of mitochondrial genome loss and has a respiratory growth defect [Source:SGD;Acc:S000006020] 0.6466557142168948 60 YHR147C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001190] 0.6462910828907357 96 YPL024W Subunit of the RecQ (Sgs1p) - Topo III (Top3p) complex; stimulates superhelical relaxing, DNA catenation/decatenation and ssDNA binding activities of Top3p; involved in response to DNA damage; functions in S phase-mediated cohesion establishment via a pathway involving the Ctf18-RFC complex and Mrc1p; stimulates Top3p DNA catenation/decatenation activity; null mutants display increased rates of recombination and delayed S phase [Source:SGD;Acc:S000005945] 0.6451485515216593 62 YMR244C-A Protein involved in cytochrome c oxidase (Complex IV) assembly; involved in delivery of copper to Complex IV; also required for efficient formation of respiratory supercomplexes comprised of Complexes III and IV; localizes to the mitochondrial intermembrane space; ortholog implicated in cardiac defects in zebrafish and human; transcription is induced in response to the DNA-damaging agent MMS; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000004857] 0.6429472491087429 63 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.6422610156260671 98 YPR005C Cytoplasmic protein involved in halotolerance; decreases intracellular Na+ (via Ena1p) and increases intracellular K+ by decreasing efflux; expression repressed by Ssn6p-Tup1p and Sko1p and induced by NaCl, KCl, and sorbitol through Gcn4p [Source:SGD;Acc:S000006209] 0.6414282529052288 66 YGR244C Beta subunit of succinyl-CoA ligase; succinyl-CoA ligase is a mitochondrial enzyme of the TCA cycle that catalyzes the nucleotide-dependent conversion of succinyl-CoA to succinate [Source:SGD;Acc:S000003476] 0.6383246262493834 69 YOR097C Putative protein of unknown function; identified as interacting with Hsp82p in a high-throughput two-hybrid screen; YOR097C is not an essential gene [Source:SGD;Acc:S000005623] 0.6368028571073604 70 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] 0.6365738320131532 71 YNL306W Mitochondrial ribosomal protein of the small subunit; essential for viability, unlike most other mitoribosomal proteins [Source:SGD;Acc:S000005250] 0.6361793112359764 72 YHR116W Protein that functions in mitochondrial copper homeostasis; mitochondrial intermembrane space protein; essential for functional cytochrome oxidase expression; homologous to Cox17p; contains twin cysteine-x9-cysteine motifs [Source:SGD;Acc:S000001158] 0.6351109786195948 74 YML087C Protein of unknown function, highly conserved across species; homolog of human CYB5R4; null mutant displays reduced frequency of mitochondrial genome loss; AIM33 has a paralog, PGA3, that arose from the whole genome duplication [Source:SGD;Acc:S000004552] 0.6334534431956121 76 YHR005C-A 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 [Source:SGD;Acc:S000003530] 0.6313278838866232 77 YDR357C Subunit of the BLOC-1 complex involved in endosomal maturation; interacts with Msb3p; null mutant is sensitive to drug inducing secretion of vacuolar cargo; green fluorescent protein (GFP)-fusion protein localizes to the cytoplasm [Source:SGD;Acc:S000002765] 0.6298744774629541 81 YIL157C Mitochondrial inner membrane protein; required for assembly of the cytochrome c oxidase complex (complex IV); interacts with complex IV assembly factor Shy1p during the early stages of assembly [Source:SGD;Acc:S000001419] 0.6284045107858559 90 YGL087C Ubiquitin-conjugating enzyme variant; involved in error-free postreplication repair; forms a heteromeric complex with Ubc13p, an active ubiquitin-conjugating enzyme; cooperates with chromatin-associated RING finger proteins, Rad18p and Rad5p; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000003055] 0.6283806830981382 84 YLR147C Core Sm protein Sm D3; part of heteroheptameric complex (with Smb1p, Smd1p, Smd2p, Sme1p, Smx3p, and Smx2p) that is part of the spliceosomal U1, U2, U4, and U5 snRNPs; homolog of human Sm D3 [Source:SGD;Acc:S000004137] 0.6259672916704845 88 YDL178W D-2-hydroxyglutarate dehydrogenase, and minor D-lactate dehydrogenase; mitochondrial matrix protein that oxidizes D-2-hydroxyglutarate (D-2HG), an oncometabolite, to alpha-ketoglutarate with a minor role in lactate catabolism; located in the mitochondrial matrix [Source:SGD;Acc:S000002337] 0.6206271962384963 90 YOR089C Endosomal Rab family GTPase; required for endocytic transport and sorting of vacuolar hydrolases; required for endosomal localization of the CORVET complex; required with YPT52 for MVB biogenesis and sorting; involved in autophagy and ionic stress tolerance; geranylgeranylation required for membrane association; protein abundance increases in response to DNA replication stress; mammalian Rab5 homolog; VPS21 has a paralog, YPT53, that arose from the whole genome duplication [Source:SGD;Acc:S000005615] 0.6204579858323015 91 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] 0.6199101669720581 93 YFL005W Rab family GTPase; essential for vesicle-mediated exocytic secretion and autophagy; associates with the exocyst component Sec15p and may regulate polarized delivery of transport vesicles to the exocyst at the plasma membrane [Source:SGD;Acc:S000001889] 0.6192229316706283 94 YJR034W Protein required for assembly of cytochrome c oxidase; exists as an oligomer; described as both an integral mitochondrial inner membrane protein facing the intermembrane space (IMS) and as a soluble IMS protein; contains a twin Cx9C motif; imported into the IMS via the MIA import machinery [Source:SGD;Acc:S000003795] 0.6162483342560003 100