Sequence Description Alias PCC hrr YNL177C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005121] 0.8324158124095465 10 YNL137C Mitochondrial ribosomal component of the small subunit [Source:SGD;Acc:S000005081] 0.8323128811964221 10 YPL173W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006094] 0.828736555778242 8 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.8205141654316727 11 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] 0.8156804973136021 14 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] 0.8090667502387392 12 YDR237W Mitochondrial ribosomal protein of the large subunit; MRPL7 produces both YmL5 and YmL7, which are two different modified forms of the same protein [Source:SGD;Acc:S000002645] 0.808510886069703 21 YGR165W Mitochondrial ribosomal protein of the small subunit; null mutant does not grow on glycerol, is sensitive to 2,4-dichlorophenol, and accumulates large lipid droplets [Source:SGD;Acc:S000003397] 0.8021526369728129 25 YJL096W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003632] 0.8010210416177665 12 YLR312W-A Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004304] 0.7935667750490496 20 YDR347W Mitochondrial ribosomal protein of the small subunit; MRP1 exhibits genetic interactions with PET122, encoding a COX3-specific translational activator, and with PET123, encoding a small subunit mitochondrial ribosomal protein [Source:SGD;Acc:S000002755] 0.7935026864767482 13 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.7928260822837235 22 YMR024W Mitochondrial ribosomal protein of the large subunit; located in close proximity to the polypeptide exit channel of the ribosome; mutations in human homolog MRPL44 cause childhood cardiomyopathy; human MRPL44 deficiency results in inefficient assembly of the mitochondrial ribosome, and in tissue-specific respiratory chain deficiency, manifesting as either Complex I+Complex IV or Complex IV deficiency, depending on a cell type [Source:SGD;Acc:S000004626] 0.7907375993724587 13 YDR322W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002730] 0.7898822971503769 17 YBR146W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000350] 0.7892359422278646 26 YDR175C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002582] 0.7869395280690233 18 YLR253W Mitochondrial protein of unknown function involved in lipid homeostasis; associates with mitochondrial ribosome; integral membrane protein that localizes to the mitochondrial inner membrane; involved in mitochondrial morphology; non-essential gene which interacts genetically with MDM10, and other members of the ERMES complex; transcription is periodic during the metabolic cycle; homologous to human aarF domain containing kinase, ADCK1 [Source:SGD;Acc:S000004243] 0.7846753136248454 17 YHR038W Mitochondrial ribosome recycling factor; essential for mitochondrial protein synthesis and for the maintenance of the respiratory function of mitochondria [Source:SGD;Acc:S000001080] 0.7842219036670404 18 YDR375C Protein translocase and chaperone required for Complex III assembly; member of the AAA ATPase family; forms a homo-oligomeric complex in the mitochondrial inner membrane that translocates the C-terminal domain of Rip1p from the matrix across the inner membrane and delivers it to an assembly intermediate of respiratory Complex III; also required for assembly of the Qcr10p subunit; mutation is functionally complemented by human homolog BCS1L, linked to neonatal diseases [Source:SGD;Acc:S000002783] 0.7705756897544186 19 YKR006C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001714] 0.7656864247644701 33 YNL315C Molecular chaperone; required for the assembly of alpha and beta subunits into the F1 sector of mitochondrial F1F0 ATP synthase; N-terminally propionylated in vivo [Source:SGD;Acc:S000005259] 0.7653603909853383 25 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] 0.7651155636483058 28 YJL180C Assembly factor for F1 sector of mitochondrial F1F0 ATP synthase; conserved protein; required for assembly of alpha and beta subunits into F1 sector of mitochondrial F1F0 ATP synthase; human homolog ATPAF2 can complement yeast atp12 mutant; mutation of human homolog reduces active ATP synthase levels and is associated with the disorder ATPAF2 deficiency [Source:SGD;Acc:S000003716] 0.7623780508805503 23 YDR494W Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation [Source:SGD;Acc:S000002902] 0.7620490015700991 39 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.7590814398442631 38 YDR337W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002745] 0.7572420030313594 26 YGL129C Mitochondrial ribosomal protein of the small subunit; has similarity to mammalian apoptosis mediator proteins; null mutation prevents induction of apoptosis by overproduction of metacaspase Mca1p [Source:SGD;Acc:S000003097] 0.7557669600872059 27 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.7553805379892051 28 YLR163C Beta subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins [Source:SGD;Acc:S000004153] 0.7509875694489863 29 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.7509574347614203 30 YHR147C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001190] 0.7500698968570451 31 YGR220C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003452] 0.7492002201448023 32 YDL202W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000002361] 0.7489158164126971 33 YGR084C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003316] 0.7420296126295141 34 YBR185C Membrane-associated mitochondrial ribosome receptor; forms a complex with Mdm38p that may facilitate recruitment of mRNA-specific translational activators to ribosomes; possible role in protein export from the matrix to inner membrane [Source:SGD;Acc:S000000389] 0.7408081192681778 35 YER087W Protein with similarity to tRNA synthetases; non-tagged protein is detected in purified mitochondria; null mutant is viable and displays elevated frequency of mitochondrial genome loss [Source:SGD;Acc:S000000889] 0.7400913786831704 36 YML025C Mitochondrial ribosomal protein of the large subunit; has similarity to E. coli L4 ribosomal protein and human mitoribosomal MRP-L4 protein; essential for viability, unlike most other mitoribosomal proteins [Source:SGD;Acc:S000004487] 0.7375278564303606 37 YBL090W Mitochondrial ribosomal protein of the small subunit; MRP21 exhibits genetic interactions with mutations in the COX2 and COX3 mRNA 5'-untranslated leader sequences [Source:SGD;Acc:S000000186] 0.7362945466135319 38 YLR239C Lipoyl ligase; involved in the modification of mitochondrial enzymes by the attachment of lipoic acid groups [Source:SGD;Acc:S000004229] 0.736107333689157 39 YBL038W Mitochondrial ribosomal protein of the large subunit; homologous to bacterial L16 ribosomal protein; synthetic lethality with hac1 mutation suggests a possible role in synthesis of precursors for protein glycosylation [Source:SGD;Acc:S000000134] 0.7348299427758749 40 YCR024C Mitochondrial asparaginyl-tRNA synthetase [Source:SGD;Acc:S000000618] 0.7340293336909656 41 YPL104W Mitochondrial aspartyl-tRNA synthetase; required for acylation of aspartyl-tRNA; yeast and bacterial aspartyl-, asparaginyl-, and lysyl-tRNA synthetases contain regions with high sequence similarity, suggesting a common ancestral gene [Source:SGD;Acc:S000006025] 0.7337064194065679 42 YPL103C Protein with a role in maintaining mitochondrial morphology; also involved in maintaining normal cardiolipin levels; mitochondrial inner membrane protein; proposed to be involved in N-acylethanolamine metabolism; related to mammalian N-acylPE-specific phospholipase D [Source:SGD;Acc:S000006024] 0.7333904252549561 43 YIL070C Specific translational activator for the mitochondrial COX1 mRNA; acidic protein of the mitochondrial matrix; related to the human complement receptor gC1q-R [Source:SGD;Acc:S000001332] 0.733282864032741 44 YBL080C Subunit of the trimeric GatFAB AmidoTransferase(AdT) complex; involved in the formation of Q-tRNAQ; mutation is functionally complemented by the bacterial GatB ortholog [Source:SGD;Acc:S000000176] 0.7330465273042905 45 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.7321907051227213 46 YNL252C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005196] 0.7310786617253597 47 YCR071C Mitochondrial ribosomal protein of the large subunit; conserved in metazoa, with similarity to human mitochondrial ribosomal protein MRPL49 [Source:SGD;Acc:S000000667] 0.7297790348907583 48 YGR171C Mitochondrial methionyl-tRNA synthetase (MetRS); functions as a monomer in mitochondrial protein synthesis; functions similarly to cytoplasmic MetRS although the cytoplasmic form contains a zinc-binding domain not found in Msm1p [Source:SGD;Acc:S000003403] 0.7286052037339851 49 YJL046W Putative lipoate-protein ligase; required along with Lip2 and Lip5 for lipoylation of Lat1p and Kgd2p; similar to E. coli LplA; null mutant displays reduced frequency of mitochondrial genome loss [Source:SGD;Acc:S000003582] 0.7275351902183963 50 YMR293C Subunit of the trimeric GatFAB AmidoTransferase(AdT) complex; involved in the formation of Q-tRNAQ; required for remodeling of ER caused by Hmg2p overexpression; similar to bacterial GatA glutamyl-tRNA amidotransferase [Source:SGD;Acc:S000004907] 0.7265396761884603 51 YGR112W Mitochondrial inner membrane protein required for complex IV assembly; associates with complex IV assembly intermediates and complex III/complex IV supercomplexes; similar to human SURF1 involved in Leigh Syndrome; complex IV is also known as cytochrome c oxidase [Source:SGD;Acc:S000003344] 0.7263322492388113 52 YCR003W Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000000596] 0.7262481979432684 53 YPL132W Protein required for delivery of copper to Cox1p; mitochondrial inner membrane protein; association with mitochondrial ribosomes suggests that copper delivery may occur during translation of Cox1p [Source:SGD;Acc:S000006053] 0.7262018835208925 54 YKL087C 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 [Source:SGD;Acc:S000001570] 0.7246111021293962 55 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] 0.7219956834044368 56 YNR040W Putative protein of unknown function; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000005323] 0.7206055121676472 57 YKL195W 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 [Source:SGD;Acc:S000001678] 0.7200848400940174 58 YHR091C Mitochondrial arginyl-tRNA synthetase; mutations in human ortholog are associated with pontocerebellar hypoplasia type 6; MSR1 has a paralog, YDR341C, that arose from the whole genome duplication [Source:SGD;Acc:S000001133] 0.7187069638765267 59 YGL143C Mitochondrial translation release factor; involved in stop codon recognition and hydrolysis of the peptidyl-tRNA bond during mitochondrial translation; lack of MRF1 causes mitochondrial genome instability [Source:SGD;Acc:S000003111] 0.7169257019754961 60 YDR316W Protein integral to the mitochondrial membrane; has a conserved methyltransferase motif and is predicted to be an RNA methyltransferase; multicopy suppressor of respiratory defects caused by OXA1 mutations [Source:SGD;Acc:S000002724] 0.7135931572818488 61 YBR044C Protein involved in assembly of the succinate dehydrogenase complex; mitochondrial; putative chaperone [Source:SGD;Acc:S000000248] 0.7124667221683246 62 YOR158W Mitochondrial ribosomal protein of the small subunit; PET123 exhibits genetic interactions with PET122, which encodes a COX3 mRNA-specific translational activator [Source:SGD;Acc:S000005684] 0.7081598362575525 63 YLR289W Mitochondrial matrix GTPase; associates with mitochondrial ribosomes; important for translation under temperature and nutrient stress; may have a role in translational fidelity; similar to bacterial LepA elongation factor [Source:SGD;Acc:S000004280] 0.707417657580404 64 YMR193W Mitochondrial ribosomal protein of the large subunit; two mitochondrial ribosomal proteins, YmL14 and YmL24, have been assigned to the same gene [Source:SGD;Acc:S000004806] 0.7065806283271499 65 YDR296W Mitochondrial ribosomal protein of the large subunit; also involved in homologous recombination in mitochondria; required for recombination-dependent mtDNA partitioning; involved in stimulation of mitochondrial DNA replication in response to oxidative stress [Source:SGD;Acc:S000002704] 0.7047664075805737 66 YBR122C Mitochondrial ribosomal protein of the large subunit; overproduction suppresses mutations in the COX2 leader peptide-encoding region [Source:SGD;Acc:S000000326] 0.7041007343475846 67 YKL194C Mitochondrial threonyl-tRNA synthetase; aminoacylates both the canonical threonine tRNA tT(UGU)Q1 and the unusual threonine tRNA tT(UAG)Q2 in vitro; lacks a typical editing domain, but has pre-transfer editing activity stimulated by the unusual tRNA-Thr [Source:SGD;Acc:S000001677] 0.7031634681345992 68 YFR007W Putative kinase with similarity to the PRK/URK/PANK kinase subfamily; the PRK/URK/PANK subfamily of P-loop kinases is also known as phosphoribulokinase/uridine kinase/bacterial pantothenate kinase [Source:SGD;Acc:S000001903] 0.7022263930503367 69 YJL063C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003599] 0.7005808531605497 70 YGL107C Mitochondrial protein required for respiratory growth; mutant phenotype and genetic interactions suggest a role in delivering mt mRNAs to ribosomes; located on matrix face of the inner membrane and loosely associated with mitoribosomes; RMD9 has a paralog, YBR238C, that arose from the whole genome duplication [Source:SGD;Acc:S000003075] 0.6999376939688973 75 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] 0.6979441799047034 72 YMR282C Mitochondrial protein; likely involved in translation of the mitochondrial OLI1 mRNA; exhibits genetic interaction with the OLI1 mRNA 5'-untranslated leader [Source:SGD;Acc:S000004895] 0.6935916784668642 73 YPR100W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006304] 0.6927863223974235 74 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.6918498137027688 75 YOR266W Mitochondrial integral inner membrane protein; involved in membrane insertion of C-terminus of Cox2p, interacts genetically and physically with Cox18p; deletion mutant sensitive to the anti-Pneumocystis carinii drug pentamidine [Source:SGD;Acc:S000005792] 0.6910008000763972 76 YOR150W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000005676] 0.6880792825929293 77 YJR122W Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system [Source:SGD;Acc:S000003883] 0.6832000997552072 78 YDR061W Protein with similarity to ABC transporter family members; lacks predicted membrane-spanning regions; transcriptionally activated by Yrm1p along with genes involved in multidrug resistance [Source:SGD;Acc:S000002468] 0.6831536102235838 79 YIL093C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000001355] 0.6769084550656944 80 YER050C Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S18 ribosomal protein [Source:SGD;Acc:S000000852] 0.6759146209446201 81 YPR047W Mitochondrial phenylalanyl-tRNA synthetase; active as a monomer, unlike the cytoplasmic subunit which is active as a dimer complexed to a beta subunit dimer; similar to the alpha subunit of E. coli phenylalanyl-tRNA synthetase [Source:SGD;Acc:S000006251] 0.6749871149583562 82 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6749387914057768 83 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] 0.6747217263996219 84 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] 0.6744555039495352 85 YLR203C Specific translational activator for the mitochondrial COX1 mRNA; loosely associated with the matrix face of the mitochondrial inner membrane; localizes to vacuole membrane in response to H2O2; influences both COX1 mRNA translation and Cox1p assembly into cytochrome c oxidase; binds to heme B, which may be a mechanism for sensing oxygen levels in order to regulate cytochrome c oxidase biogenesis [Source:SGD;Acc:S000004193] 0.6743884490377954 86 YNL131W 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 [Source:SGD;Acc:S000005075] 0.6704968646534519 88 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] 0.668292980849869 90 YJR101W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003862] 0.6655626203920596 91 YMR157C Protein of unknown function; null mutant displays reduced respiratory growth and elevated frequency of mitochondrial genome loss; the authentic, non-tagged protein is detected in purified mitochondria in high-throughput studies [Source:SGD;Acc:S000004766] 0.6651024721004524 92 YNL073W Mitochondrial lysine-tRNA synthetase; required for import of both aminoacylated and deacylated forms of tRNA(Lys) into mitochondria and for aminoacylation of mitochondrially encoded tRNA(Lys) [Source:SGD;Acc:S000005017] 0.663096152876563 93 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] 0.6617313984521668 94 YJL112W Peripheral protein of cytosolic face of mitochondrial outer membrane; required for mitochondrial fission; interacts with Fis1p and with the self-assembled oligomeric form of the dynamin-related GTPase Dnm1p; contains WD repeats; MDV1 has a paralog, CAF4, that arose from the whole genome duplication [Source:SGD;Acc:S000003648] 0.6610780406516854 95 YHL014C Protein of unknown function; has weak similarity to E. coli GTP-binding protein gtp1; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000001006] 0.660248329711888 96 YLL001W Dynamin-related GTPase involved in mitochondrial organization; required for mitochondrial fission and inheritance; self assembles on the cytoplasmic face of mitochondrial tubules at sites where division will occur; participates in endocytosis and regulates peroxisome fission along with Vps1p; mutants in the human ortholog DNM1L, which mediates mitochondrial fission, peroxisomal division, autophagy, and mitophagy, are associated with slowly progressive infantile encephalopathy [Source:SGD;Acc:S000003924] 0.659883359169555 97 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] 0.6597355293412931 98 YFR011C 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 [Source:SGD;Acc:S000001907] 0.658012191585993 99