Sequence Description Alias PCC hrr 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] 0.8003806444365336 10 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] 0.7535052049140638 6 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] 0.7426858131128179 21 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] 0.7399005142808884 6 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] 0.7398029512097777 38 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] 0.7364711461958013 53 YNL137C Mitochondrial ribosomal component of the small subunit [Source:SGD;Acc:S000005081] 0.7309634663001686 67 YBR003W Hexaprenyl pyrophosphate synthetase; catalyzes the first step in ubiquinone (coenzyme Q) biosynthesis [Source:SGD;Acc:S000000207] 0.7289861486902092 8 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.7288767324645002 56 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.728439889823614 59 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] 0.724035798053177 11 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] 0.7173906319315366 48 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] 0.7169161259930572 13 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] 0.7109701935065955 46 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] 0.71049677552449 48 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] 0.7104250327005687 52 YDR322W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002730] 0.7099840466179278 54 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] 0.7070912033923615 78 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] 0.7065634467468929 56 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] 0.7061466468567136 67 YBR146W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000350] 0.7049606529040952 66 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] 0.7024476084243643 22 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] 0.7006616643298649 23 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] 0.6943456334856378 67 YDR494W Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation [Source:SGD;Acc:S000002902] 0.6921361072677735 95 YNL177C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005121] 0.6905211583807798 100 YKR006C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001714] 0.687796062098627 79 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] 0.6877150032043353 59 YHR147C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000001190] 0.6869921899864335 69 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] 0.6869265696588618 52 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.6834864413104397 71 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] 0.6832321998007193 41 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] 0.6810562532447303 72 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] 0.6804357522830604 84 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.678530802966353 99 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] 0.6776739719593141 46 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] 0.6746772758185908 69 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] 0.6743884490377954 86 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] 0.6726686921400726 99 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.6701911708366842 78 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] 0.6700199630311746 100 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] 0.667316733762746 90 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] 0.6652088533487609 68 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] 0.6643809319447002 45 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] 0.6624995064543683 50 YGR220C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003452] 0.6599090576732938 59 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] 0.6587811570006755 57 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] 0.6490085923777019 49 YJL096W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003632] 0.6488305027928826 96 YBR044C Protein involved in assembly of the succinate dehydrogenase complex; mitochondrial; putative chaperone [Source:SGD;Acc:S000000248] 0.6477618409511194 82 YGR101W Mitochondrial serine protease; required for the processing of various mitochondrial proteins and maintenance of mitochondrial DNA and morphology; belongs to the rhomboid-GlpG superfamily of intramembrane peptidases [Source:SGD;Acc:S000003333] 0.647218305243509 52 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] 0.6440141480412074 53 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] 0.6335116991706204 57 YLR439W Mitochondrial ribosomal protein of the large subunit; homolog of prokaryotic L29 ribosomal protein; located at the ribosomal tunnel exit [Source:SGD;Acc:S000004431] 0.6321104885618043 59 YFR045W Putative mitochondrial transport protein; null mutant is viable, exhibits decreased levels of chitin and normal resistance to calcofluor white [Source:SGD;Acc:S000001941] 0.6299076844689205 62 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.6274927217128228 98 YJR045C Hsp70 family ATPase; constituent of the import motor component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); involved in protein translocation and folding; subunit of SceI endonuclease; SSC1 has a paralog, ECM10, that arose from the whole genome duplication [Source:SGD;Acc:S000003806] 0.625798500262842 68 YLR259C Tetradecameric mitochondrial chaperonin; required for ATP-dependent folding of precursor polypeptides and complex assembly; prevents aggregation and mediates protein refolding after heat shock; role in mtDNA transmission; phosphorylated [Source:SGD;Acc:S000004249] 0.6244595934840528 69 YHR198C Protein of unknown function; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies; null mutant displays elevated frequency of mitochondrial genome loss [Source:SGD;Acc:S000001241] 0.623645135977105 70 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] 0.6227148557866514 79 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] 0.6226219045352755 93 YER141W Protein required for the hydroxylation of heme O to form heme A; heme A is an essential prosthetic group for cytochrome c oxidase [Source:SGD;Acc:S000000943] 0.62179969945309 73 YLR201C Protein required for ubiquinone biosynthesis and respiratory growth; localizes to matrix face of mitochondrial inner membrane in a large complex with ubiquinone biosynthetic enzymes; ubiquinone is also known as coenzyme Q; human homolog COQ9 can complement yeast coq9 null mutant [Source:SGD;Acc:S000004191] 0.6201096233206194 74 YJL063C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000003599] 0.6171060332561663 98 YNL185C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005129] 0.6112758544333416 80 YCR046C Mitochondrial ribosomal protein of the large subunit; required for respiration and for maintenance of the mitochondrial genome [Source:SGD;Acc:S000000642] 0.6088353681005158 82 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] 0.6048869231172137 89 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] 0.6046160076932674 87 YJR113C Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S7 ribosomal protein [Source:SGD;Acc:S000003874] 0.5975347289490125 92 YDR116C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002523] 0.5956096020435064 95 YOR150W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000005676] 0.5926424115844504 100