Sequence Description Alias PCC hrr YDR175C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002582] 0.8284655694172539 3 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.8103090538108015 7 YDR494W Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation [Source:SGD;Acc:S000002902] 0.8040777912819239 17 YPL173W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006094] 0.798205561302112 24 YMR188C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000004800] 0.7970843324722251 5 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] 0.7953721731077978 6 YLR312W-A Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004304] 0.7862363453646549 24 YNL177C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005121] 0.7849487467187947 37 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] 0.7840537387757927 10 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.7838373236424321 24 YGR084C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003316] 0.7817867261124412 11 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] 0.7736429580457228 14 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.7721291319835548 36 YJL063C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003599] 0.7713783183220742 14 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] 0.7703752819937071 15 YDR405W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002813] 0.7696712241294521 16 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.7655893093393065 17 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] 0.7654449265475328 18 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] 0.7612229443328867 28 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] 0.7605371892824269 29 YIL093C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000001355] 0.7586040637153422 21 YDL202W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000002361] 0.7584862458050138 22 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] 0.758472247616614 29 YNL137C Mitochondrial ribosomal component of the small subunit [Source:SGD;Acc:S000005081] 0.7571088496945202 47 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] 0.7507423062088657 49 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] 0.75052919698126 31 YNL252C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005196] 0.7503564144330565 27 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.7502516510521972 28 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] 0.7501290367713713 38 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.7497439653616671 58 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] 0.7465514943998836 31 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] 0.7464703116368354 33 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] 0.7440036670921653 51 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] 0.7418565062384835 62 YBR146W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000350] 0.7407130832710942 48 YLR439W Mitochondrial ribosomal protein of the large subunit; homolog of prokaryotic L29 ribosomal protein; located at the ribosomal tunnel exit [Source:SGD;Acc:S000004431] 0.7401677494306041 36 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] 0.7375278564303606 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] 0.7348812324830789 38 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] 0.733643747717366 39 YCR046C Mitochondrial ribosomal protein of the large subunit; required for respiration and for maintenance of the mitochondrial genome [Source:SGD;Acc:S000000642] 0.7326011159394751 40 YHR038W Mitochondrial ribosome recycling factor; essential for mitochondrial protein synthesis and for the maintenance of the respiratory function of mitochondria [Source:SGD;Acc:S000001080] 0.7298434992002015 41 YDR322W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002730] 0.7257761164847563 44 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] 0.7257382423658617 49 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] 0.7239119462109868 44 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] 0.7237207791063086 45 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] 0.7211080423562318 46 YDR462W Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000002870] 0.7192901428309367 47 YKR006C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001714] 0.7178277407596727 59 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] 0.7163623265991069 49 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] 0.7127952484016741 50 YCR071C Mitochondrial ribosomal protein of the large subunit; conserved in metazoa, with similarity to human mitochondrial ribosomal protein MRPL49 [Source:SGD;Acc:S000000667] 0.7106966549018559 57 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] 0.7104250327005687 52 YJL096W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003632] 0.7085742964900742 55 YGR220C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003452] 0.7062300834032291 54 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] 0.705851385919078 55 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] 0.70240220079927 56 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] 0.7016380554447981 59 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] 0.700451325531472 58 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] 0.6992433380092248 59 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] 0.6981907941475082 60 YJR113C Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S7 ribosomal protein [Source:SGD;Acc:S000003874] 0.6963968905841225 61 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.6945786660021985 62 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] 0.6944490058234553 66 YPR100W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006304] 0.6933435798667573 64 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] 0.6924918791857195 65 YER050C Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S18 ribosomal protein [Source:SGD;Acc:S000000852] 0.6912911091148511 66 YLR239C Lipoyl ligase; involved in the modification of mitochondrial enzymes by the attachment of lipoic acid groups [Source:SGD;Acc:S000004229] 0.6907444469583678 83 YDL045W-A Mitochondrial ribosomal protein of the small subunit; contains twin cysteine-x9-cysteine motifs; oxidized by Mia40p during import into mitochondria [Source:SGD;Acc:S000006430] 0.6859132752341445 68 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] 0.6850724965189708 69 YBR122C Mitochondrial ribosomal protein of the large subunit; overproduction suppresses mutations in the COX2 leader peptide-encoding region [Source:SGD;Acc:S000000326] 0.6838787173707465 70 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] 0.6835050915511799 71 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] 0.6785517701338615 72 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] 0.6768664102152133 73 YER017C Mitochondrial inner membrane m-AAA protease component; mediates degradation of misfolded or unassembled proteins; also required for correct assembly of mitochondrial enzyme complexes; involved in cytoplasmic mRNA translation and aging; expression of human homolog AFG3L2 can complement yeast yta12 afg3 double mutant [Source:SGD;Acc:S000000819] 0.6742169835303741 74 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] 0.6705711414289349 75 YCR003W Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000000596] 0.6690109453128695 77 YGL219C Mitochondrial component of the ERMES complex; links the ER to mitochondria and may promote inter-organellar calcium and phospholipid exchange as well as coordinating mitochondrial DNA replication and growth; required for mitophagy; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase [Source:SGD;Acc:S000003187] 0.6676360079517256 85 YMR166C Transporter of the mitochondrial inner membrane that exports magnesium; involved in mitochondrial Mg2+ homeostasis; has similarity to human mitochondrial ATP-Mg/Pi carriers [Source:SGD;Acc:S000004776] 0.6622839493722138 80 YHR147C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001190] 0.6618542928506495 86 YDR337W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002745] 0.6614566788029819 82 YER058W Protein required for assembly of cytochrome c oxidase [Source:SGD;Acc:S000000860] 0.6592538195799527 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] 0.6536126613751811 84 YIL022W Essential component of the TIM23 complex; tethers the import motor and regulatory factors (PAM complex) to the translocation channel (Tim23p-Tim17p core complex); TIM23 complex is short for the translocase of the inner mitochondrial membrane [Source:SGD;Acc:S000001284] 0.6534658334534392 85 YMR002W Mitochondrial intermembrane space protein; required for normal oxygen consumption; contains twin cysteine-x9-cysteine motifs; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000004604] 0.6529098476496673 86 YDR116C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002523] 0.6524529920047313 87 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] 0.6500010207706862 93 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] 0.6497912680659613 90 YLR382C Mitochondrial leucyl-tRNA synthetase; also has direct role in splicing of several mitochondrial group I introns; indirectly required for mitochondrial genome maintenance; human homolog LARS2 can complement yeast null mutant, and is implicated in Perrault syndrome [Source:SGD;Acc:S000004374] 0.6477035167126176 91 YNL185C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005129] 0.6456670548495659 92 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] 0.6456017054405835 93 YNL081C Putative mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S13 ribosomal protein; participates in controlling sporulation efficiency; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000005025] 0.64254067752714 96 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] 0.6417616124321489 97 YKL138C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001621] 0.6416893471491333 98 YBR192W Mitochondrial pyrimidine nucleotide transporter; imports pyrimidine nucleoside triphosphates and exports pyrimidine nucleoside monophosphates; member of the mitochondrial carrier family [Source:SGD;Acc:S000000396] 0.6403027888541268 99 YGL236C Mitochondrial protein; forms heterodimer complex with Mss1p that performs 5-carboxymethylaminomethyl modification of the wobble uridine base in mitochondrial tRNAs; required for respiration in paromomycin-resistant 15S rRNA mutants; human homolog MTO1 can complement yeast null mutant [Source:SGD;Acc:S000003205] 0.639150265513776 100