Sequence Description Alias PCC hrr 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] 0.8490759272391163 5 YHR147C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001190] 0.8455497354812028 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] 0.841592027471721 3 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] 0.8247689430390204 4 YLR312W-A Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004304] 0.814641427022711 11 YDR322W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002730] 0.8125701823500505 11 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] 0.8111311734884515 17 YBR146W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000350] 0.8102606801573998 17 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] 0.8072672128902874 13 YNL177C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005121] 0.8037894008251909 22 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] 0.8021210596173532 11 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] 0.8010210416177665 12 YPL173W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006094] 0.7969534229602706 26 YPR100W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006304] 0.7933112647659427 14 YGR220C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003452] 0.7839123955155792 15 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] 0.7824174998754317 19 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.7769896691593123 34 YCR071C Mitochondrial ribosomal protein of the large subunit; conserved in metazoa, with similarity to human mitochondrial ribosomal protein MRPL49 [Source:SGD;Acc:S000000667] 0.7747427897422351 19 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.7744344751013779 33 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] 0.7734472268979482 25 YPR166C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000006370] 0.772437035489059 21 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] 0.7712638269283113 22 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] 0.7706124709510599 30 YHR038W Mitochondrial ribosome recycling factor; essential for mitochondrial protein synthesis and for the maintenance of the respiratory function of mitochondria [Source:SGD;Acc:S000001080] 0.7703009475352618 24 YKR006C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001714] 0.7698509699880752 30 YDR337W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002745] 0.7693766157255324 26 YJR101W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003862] 0.7652406721545508 27 YCR003W Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000000596] 0.7639265157610174 28 YDR494W Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation [Source:SGD;Acc:S000002902] 0.7638333031228317 37 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] 0.757407678684226 30 YOR150W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000005676] 0.7557853567833513 31 YNL137C Mitochondrial ribosomal component of the small subunit [Source:SGD;Acc:S000005081] 0.7528842181684374 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] 0.7527392829426 33 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] 0.7514860472812023 34 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.7506750998839391 43 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] 0.7475872566960913 36 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] 0.7456059540678719 37 YDR175C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002582] 0.7419357121415776 39 YDL202W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000002361] 0.7392466322338784 39 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] 0.7380438605029475 40 YJL063C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003599] 0.7374828625539966 41 YBR122C Mitochondrial ribosomal protein of the large subunit; overproduction suppresses mutations in the COX2 leader peptide-encoding region [Source:SGD;Acc:S000000326] 0.7322824619325762 42 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] 0.7309454387826495 45 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] 0.7303437359194882 44 YNL252C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005196] 0.7288262463238429 45 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] 0.7277648640128479 46 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] 0.7276438793239881 47 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] 0.7207601261078799 48 YNR022C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000005305] 0.7197534213540613 49 YGR084C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003316] 0.7152837050158598 50 YER058W Protein required for assembly of cytochrome c oxidase [Source:SGD;Acc:S000000860] 0.7127880252749047 51 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] 0.7115445998062455 52 YGR076C Mitochondrial ribosomal protein of the large subunit; mutation confers increased replicative lifespan [Source:SGD;Acc:S000003308] 0.7111406425240986 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] 0.7104176470852219 54 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] 0.7085742964900742 55 YKL003C Mitochondrial ribosomal protein of the small subunit; MRP17 exhibits genetic interactions with PET122, encoding a COX3-specific translational activator [Source:SGD;Acc:S000001486] 0.707467816424575 56 YER050C Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S18 ribosomal protein [Source:SGD;Acc:S000000852] 0.7058641651969889 57 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] 0.7036149226278718 58 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] 0.7035392302654112 59 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.7014933038058675 60 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.7014038367767496 61 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] 0.7000767358687253 62 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] 0.698555955200037 67 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] 0.692431416622943 64 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] 0.691231249767866 65 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] 0.6883009590125682 66 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] 0.6870975556555082 67 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] 0.6865075919646884 72 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] 0.686298891040369 69 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] 0.6847235842064454 70 YMR225C Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000004838] 0.6824488211822329 71 YIL093C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000001355] 0.6789459026203871 72 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] 0.6780133063336758 92 YNL185C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005129] 0.6773642372826143 74 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] 0.6763683810002079 75 YKL167C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001650] 0.6752885476381824 76 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] 0.6752169367233359 77 YER182W Putative protein of unknown function; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000000984] 0.6748251475490452 78 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] 0.674767382156119 79 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] 0.6726284727157565 80 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] 0.6714752640324968 81 YEL020W-A Essential protein of the mitochondrial intermembrane space; forms a complex with Tim10p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane [Source:SGD;Acc:S000007256] 0.6714239449290659 82 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] 0.6692045465761499 83 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.6653942847521795 85 YMR188C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000004800] 0.6637535187948553 85 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] 0.662956321237166 86 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] 0.6628929141547016 87 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] 0.6610420569689385 88 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] 0.6581732343351047 94 YDR116C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002523] 0.6571307033321308 90 YKL138C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001621] 0.6570999107860429 91 YCR046C Mitochondrial ribosomal protein of the large subunit; required for respiration and for maintenance of the mitochondrial genome [Source:SGD;Acc:S000000642] 0.6566170225808702 92 YGR021W Putative protein of unknown function; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000003253] 0.654874975474926 94 YML050W Protein of unknown function; null mutant is viable and displays elevated frequency of mitochondrial genome loss [Source:SGD;Acc:S000004514] 0.6511214523383967 95 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] 0.6488305027928826 96 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] 0.648556139317808 97 YNL122C Mitochondrial ribosomal protein of the large subunit; homologous to bacterial L35 and human MRPL35 ribosomal proteins [Source:SGD;Acc:S000005066] 0.645574960331648 98