Sequence Description Alias PCC hrr 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] 0.7158190093053657 47 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] 0.7134961667083767 34 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] 0.7133011090048579 42 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] 0.7077132336957398 46 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] 0.7051182681824836 46 YPL072W Deubiquitinating enzyme anchored to the outer mitochondrial membrane; probably not important for general mitochondrial functioning, but may perform a more specialized function at mitochondria [Source:SGD;Acc:S000005993] 0.7045622362495085 59 YGR169C tRNA:pseudouridine synthase; catalyzes the conversion of uridine to pseudouridine at position 31 in cytoplasmic and mitochondrial tRNAs; mutation of Asp168 to Ala abolishes enzyme activity; not essential for viability [Source:SGD;Acc:S000003401] 0.6955882344553705 7 YJL054W Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane [Source:SGD;Acc:S000003590] 0.6925695137441448 24 YPL029W ATP-dependent RNA helicase; component of the mitochondrial degradosome along with the RNase Dss1p; the degradosome associates with the ribosome and mediates RNA turnover; also required during splicing of the COX1 AI5_beta intron; expression of a processed form of human homolog SUPV3L1 carrying an N-terminal deletion of 46 amino acids rescues yeast suv3 null mutant [Source:SGD;Acc:S000005950] 0.6894956306121889 59 YLR239C Lipoyl ligase; involved in the modification of mitochondrial enzymes by the attachment of lipoic acid groups [Source:SGD;Acc:S000004229] 0.688528680033606 87 YKL155C Mitochondrial ribosomal protein of the small subunit; also predicted to be an S-adenosylmethionine-dependent RNA methyltransferase [Source:SGD;Acc:S000001638] 0.6875610914754554 90 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] 0.6845514676416348 85 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.6822156390941688 93 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] 0.6814725581052072 56 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] 0.6783698542621981 59 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] 0.6764286317626786 64 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] 0.6740393351390828 69 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.6693260509614656 76 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] 0.6636521188805116 97 YGL064C Mitochondrial ATP-dependent RNA helicase of the DEAD-box family; required for assembly of the large subunit of mitochondrial ribosomes; binds to the large subunit rRNA, 21S_rRNA; localizes to the matrix face of the mitochondrial inner membrane and associates with the large subunit precursor and with mature ribosomes [Source:SGD;Acc:S000003032] 0.6635155868916829 65 YPL040C Mitochondrial isoleucyl-tRNA synthetase; null mutant is deficient in respiratory growth; human homolog IARS2 implicated in mitochondrial diseases, can partially complement yeast null mutant [Source:SGD;Acc:S000005961] 0.6611216741978956 72 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] 0.660248329711888 96 YGR222W Mitochondrial inner membrane protein; binds to the 5' UTR of the COX3 mRNA to activate its translation together with Pet122p and Pet494p; also binds to the COX1 Group I intron AI5 beta to facilitate exon ligation during splicing [Source:SGD;Acc:S000003454] 0.6591474540566026 46 YCR024C Mitochondrial asparaginyl-tRNA synthetase [Source:SGD;Acc:S000000618] 0.657709898611013 97 YGR084C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003316] 0.6565054421071431 85 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] 0.6563146218814251 92 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] 0.655865656336736 52 YPL039W Putative protein of unknown function; YPL039W is not an essential gene [Source:SGD;Acc:S000005960] 0.6503928909054348 39 YNR020C Putative metalloprotease of the mitochondrial inner membrane; required for processing of Atp6p; has an additional role in assembly of the F0 sector of the F1F0 ATP synthase complex; substrate of the Mia40p-Erv1p disulfide relay system, and folding is assisted by Mia40p [Source:SGD;Acc:S000005303] 0.6491886272221992 40 YBR192W Mitochondrial pyrimidine nucleotide transporter; imports pyrimidine nucleoside triphosphates and exports pyrimidine nucleoside monophosphates; member of the mitochondrial carrier family [Source:SGD;Acc:S000000396] 0.6405371574558572 48 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] 0.6375554187040369 70 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6301571702889027 70 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] 0.6293305559736447 79 YOR201C Ribose methyltransferase; modifies a functionally critical, conserved nucleotide in mitochondrial 21S rRNA [Source:SGD;Acc:S000005727] 0.619270452265085 77 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] 0.6182243543608653 79