Sequence Description Alias PCC hrr 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] 0.7449350347643433 16 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] 0.7299277108346036 46 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] 0.7295896317840477 19 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] 0.7295787096520099 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] 0.7265396761884603 51 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] 0.7209446730790345 42 YNL177C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005121] 0.7194484777093627 79 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.7174143870778242 74 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.7157354394055243 68 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] 0.7040243027895152 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] 0.6923432294308198 55 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] 0.6912771944231813 77 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] 0.6883773514485259 52 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] 0.6883509696356616 27 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] 0.687584079282925 71 YHR038W Mitochondrial ribosome recycling factor; essential for mitochondrial protein synthesis and for the maintenance of the respiratory function of mitochondria [Source:SGD;Acc:S000001080] 0.6871525793019794 56 YBR044C Protein involved in assembly of the succinate dehydrogenase complex; mitochondrial; putative chaperone [Source:SGD;Acc:S000000248] 0.6864753771293329 33 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] 0.6856144649836383 88 YDR322W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002730] 0.6850182308776668 66 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] 0.6842899450927852 68 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] 0.6825959060983984 34 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] 0.6816717198673108 70 YCR024C Mitochondrial asparaginyl-tRNA synthetase [Source:SGD;Acc:S000000618] 0.6796978124060354 59 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] 0.6764353700168183 86 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] 0.6738287536405101 42 YPL173W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006094] 0.6732329040437526 90 YDR175C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002582] 0.6720454349168706 85 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] 0.6715910375646612 81 YJL096W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003632] 0.6714752640324968 81 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] 0.6698957291913017 82 YDR337W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002745] 0.6696172694867171 74 YML050W Protein of unknown function; null mutant is viable and displays elevated frequency of mitochondrial genome loss [Source:SGD;Acc:S000004514] 0.6682443328507367 36 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] 0.6680088454271793 79 YBR146W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000350] 0.6670744599213816 97 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.6658806843795431 65 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] 0.664452280267198 59 YPR100W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006304] 0.6639211298827615 58 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] 0.6634802352257637 94 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] 0.6626676016602864 77 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] 0.662650038668204 79 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] 0.6609669446897112 45 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.6603520376444654 87 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] 0.6598902036465967 56 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] 0.659048610236373 61 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] 0.6578772117581704 94 YJR100C Mitochondria protein of unknown function; interacts genetically with TOR1 to regulate chronological lifespan, and the response to both heat shock and oxidative stress; involved in maintaining the integrity of the mitochondrial network; negative regulator of mitophagy flux; non-tagged protein is detected in purified mitochondria in high-throughput studies; null mutant is viable and displays an elevated frequency of mitochondrial genome loss; similar to murine NOR1 [Source:SGD;Acc:S000003861] 0.6572601505782665 50 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] 0.6567310672851627 67 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] 0.655865656336736 52 YLR312W-A Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004304] 0.6558503491275657 90 YDR004W Protein that stimulates strand exchange; stimulates strand exchange by stabilizing the binding of Rad51p to single-stranded DNA; involved in the recombinational repair of double-strand breaks in DNA during vegetative growth and meiosis; forms heterodimer with Rad55p [Source:SGD;Acc:S000002411] 0.6548916631407459 86 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6548499718656 55 YDR282C Mitochondrial inner membrane protein of unknown function; associates with mitochondrial ribosome; localizes to the inner membrane with the C terminus facing the intermembrane space; ortholog of human RMND1, mutation in which is implicated in infantile encephaloneuromyopathy and defective mitochondrial translation [Source:SGD;Acc:S000002690] 0.6547255390571944 56 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.6543627839462971 93 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] 0.6536737230103207 58 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] 0.6523259748179858 59 YPL139C Component of both the Rpd3S and Rpd3L histone deacetylase complexes; negative regulator of meiosis; required for repression of a subset of meiotic genes during vegetative growth, binding of histone deacetylase Rpd3p required for activity, contains a NEE box and a WD repeat motif; homologous with Wtm1p; UME1 has a paralog, WTM2, that arose from the whole genome duplication [Source:SGD;Acc:S000006060] 0.6483913911348514 61 YOR150W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000005676] 0.6479029734017336 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] 0.6459480478633519 100 YMR023C Mitochondrial protein; forms a heterodimer complex with Mto1p that performs the 5-carboxymethylaminomethyl modification of the wobble uridine base in mitochondrial tRNAs; similar to human GTPBP3 [Source:SGD;Acc:S000004625] 0.6447550678562283 66 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] 0.6428798079293299 95 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] 0.6417616124321489 97 YGR163W Subunit of a TORC1-stimulating GTPase complex; subunit of the Gtr1-Gtr2 GTPase complex that stimulates TORC1 in response to amino acid stimulation; stimulates the GTPase activity of Gtr1p; negatively regulates the Ran/Tc4 GTPase cycle; activates transcription; tethered to the vacuolar membrane as part of the EGO complex (EGOC); required for sorting of Gap1p; activated by the the Lst4p-Lst7p GAP complex; localizes to cytoplasm and to chromatin; homolog of human RagC and [Source:SGD;Acc:S000003395] 0.6400358337570549 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] 0.6373419417331847 100 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] 0.6366065901011742 88 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] 0.6365904548991722 93 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] 0.6362624312937649 76 YGR220C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003452] 0.636211254139529 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] 0.6353839567699042 79 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] 0.6316277529034655 83 YFR045W Putative mitochondrial transport protein; null mutant is viable, exhibits decreased levels of chitin and normal resistance to calcofluor white [Source:SGD;Acc:S000001941] 0.6287391338728748 85 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] 0.6223212092691305 96 YDR439W Nucleolar protein that forms a complex with Csm1p; and then Mam1p at kinetochores during meiosis I to mediate accurate homolog segregation; required for condensin recruitment to the replication fork barrier site and rDNA repeat segregation [Source:SGD;Acc:S000002847] 0.622164226618174 99 YOL003C Palmitoyltransferase with autoacylation activity; required for palmitoylation of amino acid permeases containing a C-terminal Phe-Trp-Cys site; required for modification of Chs3p; member of the DHHC family of putative palmitoyltransferases [Source:SGD;Acc:S000005363] 0.622041211772849 90 YBR003W Hexaprenyl pyrophosphate synthetase; catalyzes the first step in ubiquinone (coenzyme Q) biosynthesis [Source:SGD;Acc:S000000207] 0.6196872953549702 92 YNL214W Membrane peroxin of the peroxisomal importomer complex; complex facilitates the import of peroxisomal matrix proteins; required for peroxisome biogenesis [Source:SGD;Acc:S000005158] 0.6167620605719831 96 YBR122C Mitochondrial ribosomal protein of the large subunit; overproduction suppresses mutations in the COX2 leader peptide-encoding region [Source:SGD;Acc:S000000326] 0.6144596907462537 96 YGL085W Putative protein of unknown function; mutant has long chronological lifespan; has homology to Staphylococcus aureus nuclease; GFP-fusion protein localizes to mitochondria; is induced in response to the DNA-damaging agent MMS [Source:SGD;Acc:S000003053] 0.6129499321341336 98