Sequence Description Alias PCC hrr YDR322W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002730] 0.8078347558390061 12 YJR101W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003862] 0.8064710252070922 2 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] 0.7859975655315927 5 YJL096W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003632] 0.7839123955155792 15 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.782938201842451 5 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.7797536735385929 30 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] 0.7758921622431392 13 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] 0.7757124975791626 36 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] 0.7740103353538474 21 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] 0.7681374109931843 10 YPL173W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006094] 0.7680309678587647 40 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] 0.7647436060109148 26 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] 0.763428782569575 13 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] 0.7620585345018075 44 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] 0.7588718778366228 15 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.7581864821726712 39 YDR116C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002523] 0.7540098512316523 17 YHR147C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001190] 0.751427677435582 29 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] 0.7492002201448023 32 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] 0.7455635490047464 42 YLR312W-A Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004304] 0.7455534368641287 45 YDR337W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002745] 0.7412049598905819 31 YPR166C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000006370] 0.7384959927226041 23 YDR175C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002582] 0.7364308820263938 44 YNL177C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005121] 0.7355632435128161 70 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] 0.729874762500973 26 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] 0.7297629718132699 36 YOR150W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000005676] 0.7239123554646671 28 YGL069C Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data; not conserved in closely related Saccharomyces species; 92% of ORF overlaps the uncharacterized ORF YGL068W; deletion in cyr1 mutant results in loss of stress resistance [Source:SGD;Acc:S000003037] 0.7238003454136873 29 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] 0.7203367389961768 49 YDL202W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000002361] 0.7185204150682513 39 YNL252C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005196] 0.7179596632708609 42 YHR038W Mitochondrial ribosome recycling factor; essential for mitochondrial protein synthesis and for the maintenance of the respiratory function of mitochondria [Source:SGD;Acc:S000001080] 0.7176320578728491 40 YKL003C Mitochondrial ribosomal protein of the small subunit; MRP17 exhibits genetic interactions with PET122, encoding a COX3-specific translational activator [Source:SGD;Acc:S000001486] 0.7175115796864107 34 YBR146W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000350] 0.7158051330449828 62 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] 0.7147825453568385 53 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.7111187991996747 80 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] 0.7110548493346883 52 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] 0.7082236259080471 77 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] 0.7075222919372203 50 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] 0.7062300834032291 54 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.6981324623175077 56 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] 0.697759171426118 57 YNR022C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000005305] 0.6977319865103476 44 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] 0.6969108893094551 49 YKL167C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001650] 0.6898738911024633 46 YDR494W Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation [Source:SGD;Acc:S000002902] 0.6888631901424526 98 YGL068W Mitochondrial ribosomal protein of the large subunit; has similarity to E. coli L7/L12 and human MRPL7 ribosomal proteins; associates with the mitochondrial nucleoid; required for normal respiratory growth [Source:SGD;Acc:S000003036] 0.6870011120230523 48 YMR225C Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000004838] 0.6826339757124059 50 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] 0.6813322903321759 52 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] 0.674944972185065 52 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] 0.6724434098387044 53 YKR006C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001714] 0.6713275638189583 86 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] 0.6703852462382162 55 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] 0.6698814602842074 56 YMR267W Mitochondrial inorganic pyrophosphatase; required for mitochondrial function and possibly involved in energy generation from inorganic pyrophosphate; human ortholog, PPA2, functionally complements the null mutant; mutations in human PPA2 cause a mitochondrial disease resulting in sudden unexpected cardiac arrest in infants [Source:SGD;Acc:S000004880] 0.6625304756334209 57 YBR122C Mitochondrial ribosomal protein of the large subunit; overproduction suppresses mutations in the COX2 leader peptide-encoding region [Source:SGD;Acc:S000000326] 0.6624531442620596 58 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] 0.6599090576732938 59 YPR100W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006304] 0.659682891567126 60 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] 0.6578437129213276 62 YDR511W Mitochondrial protein involved in assembly of succinate dehydrogenase; has a role in maturation of the Sdh2p subunit; localized to the mitochondrial intermembrane space; required for acetate utilization and gluconeogenesis; mutation in Drosophila ortholog SDHAF3 causes reduced succinate dehydrogenase activity and neuronal and muscular dysfunction; member of the LYR protein family [Source:SGD;Acc:S000002919] 0.6557064988185252 63 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] 0.6546173824845074 95 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] 0.65335653943867 78 YKL170W Mitochondrial ribosomal protein of the large subunit; appears as two protein spots (YmL34 and YmL38) on two-dimensional SDS gels; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000001653] 0.6484482304190927 66 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] 0.647106563698667 68 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.6443176854013003 95 YJL063C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003599] 0.6367522412036398 86 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] 0.636211254139529 77 YGR076C Mitochondrial ribosomal protein of the large subunit; mutation confers increased replicative lifespan [Source:SGD;Acc:S000003308] 0.6353216921268078 72 YMR286W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004899] 0.6257200999861258 77 YIL098C Mitochondrial matrix protein; required for assembly or stability at high temperature of the F1 sector of mitochondrial F1F0 ATP synthase; null mutant temperature sensitive growth on glycerol is suppressed by multicopy expression of Odc1p [Source:SGD;Acc:S000001360] 0.6251008388216696 78 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] 0.6222217105114658 87 YNL185C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005129] 0.6212790628104736 81 YIL093C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000001355] 0.6170856333390159 92 YBR037C Copper-binding protein of mitochondrial inner membrane; required for cytochrome c oxidase activity and respiration; may function to deliver copper to cytochrome c oxidase; similar to thioredoxins; SCO1 has a paralog, SCO2, that arose from the whole genome duplication [Source:SGD;Acc:S000000241] 0.6134984327465731 87 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6112248288439495 89 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] 0.6076420112409561 94 YJR113C Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S7 ribosomal protein [Source:SGD;Acc:S000003874] 0.5982913366725529 93 YKL138C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001621] 0.5978672188623071 94 YLR439W Mitochondrial ribosomal protein of the large subunit; homolog of prokaryotic L29 ribosomal protein; located at the ribosomal tunnel exit [Source:SGD;Acc:S000004431] 0.5966735575839718 95 YHR024C Alpha subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins [Source:SGD;Acc:S000001066] 0.5951353150256155 96 YMR188C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000004800] 0.594319195402882 98 YLR204W Mitochondrial inner membrane protein; required for accumulation of spliced COX1 mRNA; may have an additional role in translation of COX1 mRNA [Source:SGD;Acc:S000004194] 0.5938877230370794 99 YPR011C Mitochondrial transporter; major substrates are adenosine 5'-phosphosulfate (APS) and 3'-phospho-adenosine 5'-phosphosulfate (PAPS); member of the mitochondrial carrier family; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000006215] 0.5929576249294661 100