Sequence Description Alias PCC hrr 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] 0.7620446110203688 18 YDR430C Lysine-specific metalloprotease of the pitrilysin family; metalloprotease of the intermembrane space; degrades proteins and presequence peptides cleaved from imported proteins; required for normal mitochondrial morphology [Source:SGD;Acc:S000002838] 0.7569680297178266 8 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] 0.7502993823538624 32 YGL107C Mitochondrial protein required for respiratory growth; mutant phenotype and genetic interactions suggest a role in delivering mt mRNAs to ribosomes; located on matrix face of the inner membrane and loosely associated with mitoribosomes; RMD9 has a paralog, YBR238C, that arose from the whole genome duplication [Source:SGD;Acc:S000003075] 0.7484340267607225 23 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] 0.7367235299132043 25 YCR024C Mitochondrial asparaginyl-tRNA synthetase [Source:SGD;Acc:S000000618] 0.7237963528872089 22 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] 0.7196165426295051 13 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.7120795207688114 79 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.7110368612869685 64 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.7084463170604954 48 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] 0.7062019318723496 26 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] 0.7058037572194558 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] 0.6962253098857946 63 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] 0.6949278062339626 70 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] 0.6945524467368525 48 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] 0.693398562321031 16 YNL137C Mitochondrial ribosomal component of the small subunit [Source:SGD;Acc:S000005081] 0.6917033043397116 98 YNL252C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005196] 0.6884459315372712 55 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] 0.6873344307861449 30 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] 0.6861816668039286 20 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.6849031717420412 58 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] 0.6818185559670966 74 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6801031419235464 32 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] 0.6772243440966078 79 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] 0.6748337372692548 69 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] 0.6727335697070677 71 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] 0.6713753713937752 95 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] 0.6706689599590965 80 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] 0.6702129629898527 71 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] 0.6696734609965842 90 YMR089C Mitochondrial inner membrane m-AAA protease component; mediates degradation of misfolded or unassembled proteins; also required for correct assembly of mitochondrial enzyme complexes; overexpression of human AFG3L2 complements respiratory defect of yeast afg3 yta12 double null mutation, but overexpression of disease-associated AFG3L2 variants does not; expression of both human SPG7 (paraplegin) and AFG3L2 complements yeast yta12 afg3 double mutation [Source:SGD;Acc:S000004695] 0.6653564922893834 74 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] 0.6629251283730118 84 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] 0.6628291345123257 58 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] 0.6622839493722138 80 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] 0.6611590935766934 40 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.6601585956610505 87 YBR003W Hexaprenyl pyrophosphate synthetase; catalyzes the first step in ubiquinone (coenzyme Q) biosynthesis [Source:SGD;Acc:S000000207] 0.6598221407711042 42 YPL172C Heme A:farnesyltransferase; catalyzes first step in conversion of protoheme to heme A prosthetic group required for cytochrome c oxidase activity; human ortholog COX10 can complement yeast cox10 null mutant; human ortholog COX10 is associated with mitochondrial disorders [Source:SGD;Acc:S000006093] 0.6597396239375862 43 YNR033W Para-aminobenzoate (PABA) synthase; has similarity to Escherichia coli PABA synthase components PabA and PabB; required for the synthesis of para-aminobenzoic acid, an important intermediate for folate and ubiquinone Q biosynthesis; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000005316] 0.6440764735360746 50 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] 0.6440141480412074 53 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.6403540748527159 85 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] 0.6367049947952305 91 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] 0.6293282195904558 83 YBR192W Mitochondrial pyrimidine nucleotide transporter; imports pyrimidine nucleoside triphosphates and exports pyrimidine nucleoside monophosphates; member of the mitochondrial carrier family [Source:SGD;Acc:S000000396] 0.6287264280613662 61 YDL202W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000002361] 0.6140296526208027 100 YLR439W Mitochondrial ribosomal protein of the large subunit; homolog of prokaryotic L29 ribosomal protein; located at the ribosomal tunnel exit [Source:SGD;Acc:S000004431] 0.6088111924020958 85 YIL093C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000001355] 0.605617020003964 99 YLR090W Chaperone with a role in facilitating mitochondrial protein import; ascomycete-specific member of the DnaJ-like family, closely related to Ydj1p; predicted to be C-terminally prenylated; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000004080] 0.597868776931774 94 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] 0.5964524875289852 100