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] 0.8422920611798254 1 YDR175C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002582] 0.7877849551003847 17 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] 0.7804091502475018 16 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] 0.7794247420429428 11 YPL097W Mitochondrial tyrosyl-tRNA synthetase [Source:SGD;Acc:S000006018] 0.7762600622421691 36 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] 0.7732287123945778 6 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] 0.7655893093393065 17 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] 0.7629865833874737 8 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.762247449477207 19 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] 0.7574572269617832 20 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.7560153908341758 41 YGR150C Mitochondrial 15S rRNA-binding protein; required for intron removal of COB and COX1 pre-mRNAs; has separable roles in stabilizing mitochondrial 15S rRNA and in maturation of the COB and COX1 mRNAs; contains pentatricopeptide repeat (PPR) motifs; mutant is respiratory deficient and has defective plasma membrane electron transport [Source:SGD;Acc:S000003382] 0.7556915691473479 17 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.7543004311084784 45 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] 0.7508229602446778 14 YNL137C Mitochondrial ribosomal component of the small subunit [Source:SGD;Acc:S000005081] 0.749966165723979 54 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] 0.7365382373187993 34 YNL177C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005121] 0.7326317715318517 73 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] 0.7311243737186313 45 YDR494W Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation [Source:SGD;Acc:S000002902] 0.728344729906775 67 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] 0.7279945390729563 47 YDL044C Mitochondrial protein that interacts with mitochondrial RNA polymerase; interacts with an N-terminal region of mitochondrial RNA polymerase (Rpo41p) and couples RNA processing and translation to transcription [Source:SGD;Acc:S000002202] 0.7240652330238885 62 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] 0.7237273819319422 23 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] 0.723052787353445 33 YMR098C Mitochondrial protein required for the stability of Oli1p (Atp9p) mRNA; also required for the Oli1p ring formation; YMR098C is not an essential gene [Source:SGD;Acc:S000004704] 0.7223756295856832 63 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] 0.7203023970415349 27 YGR084C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003316] 0.7202740726601334 38 YBR251W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000455] 0.7175771561772154 38 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] 0.7170300911355738 37 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] 0.7139857048380196 31 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] 0.7107765532506229 32 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] 0.7106914138685632 63 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] 0.7098523973696368 34 YKL155C Mitochondrial ribosomal protein of the small subunit; also predicted to be an S-adenosylmethionine-dependent RNA methyltransferase [Source:SGD;Acc:S000001638] 0.7096015759352988 46 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] 0.7085649972173286 49 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] 0.7082951610357793 76 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] 0.7073455668580539 58 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] 0.707202097399215 52 YDR393W Protein required for normal mitochondrial morphology; mitochondrial inner membrane protein; may be involved in fission of the inner membrane; forms a homo-oligomeric complex [Source:SGD;Acc:S000002801] 0.7064116389578051 40 YPL173W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000006094] 0.703840957013444 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] 0.7033881765381235 53 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] 0.7011906448626498 49 YNL252C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000005196] 0.6999653891760339 52 YDR515W RNA binding protein that associates with polysomes; may be involved in regulating mRNA translation; involved in the copper-dependent mineralization of copper sulfide complexes on cell surface in cells cultured in copper salts; SLF1 has a paralog, SRO9, that arose from the whole genome duplication; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000002923] 0.6979920530720686 46 YMR188C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000004800] 0.6969186905572494 47 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.6939324541171618 58 YHR038W Mitochondrial ribosome recycling factor; essential for mitochondrial protein synthesis and for the maintenance of the respiratory function of mitochondria [Source:SGD;Acc:S000001080] 0.691976808752707 53 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] 0.6918498137027688 75 YIL093C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000001355] 0.6902766763495002 51 YLR239C Lipoyl ligase; involved in the modification of mitochondrial enzymes by the attachment of lipoic acid groups [Source:SGD;Acc:S000004229] 0.6902232422054978 85 YLR312W-A Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004304] 0.6898640863732008 64 YCR024C Mitochondrial asparaginyl-tRNA synthetase [Source:SGD;Acc:S000000618] 0.6885607779103439 54 YBR146W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000350] 0.6883990252860431 82 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] 0.6859454688874748 87 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] 0.6855876124691 57 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] 0.6849031717420412 58 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] 0.6843987101866282 59 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] 0.683728414268074 60 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] 0.6828725028871299 66 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] 0.6823559342843379 63 YBR179C Mitofusin; integral membrane protein involved in mitochondrial outer membrane tethering and fusion; role in mitochondrial genome maintenance; efficient tethering and degradation of Fzo1p requires an intact N-terminal GTPase domain; targeted for destruction by the ubiquitin ligase SCF-Mdm30p and the cytosolic ubiquitin-proteasome system [Source:SGD;Acc:S000000383] 0.6814732745803835 99 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] 0.6810206655215585 81 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] 0.6805686811010377 65 YDR337W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000002745] 0.6798465709422782 66 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] 0.6795471303732925 67 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] 0.6782178646867258 69 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] 0.6771314793989778 70 YKR006C Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation [Source:SGD;Acc:S000001714] 0.67291362116644 85 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] 0.6725549767073608 73 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6709161585555328 74 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] 0.6693260509614656 76 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] 0.6653848335159203 81 YBR192W Mitochondrial pyrimidine nucleotide transporter; imports pyrimidine nucleoside triphosphates and exports pyrimidine nucleoside monophosphates; member of the mitochondrial carrier family [Source:SGD;Acc:S000000396] 0.6630618104800818 82 YHR011W Probable mitochondrial seryl-tRNA synthetase; mutant displays increased invasive and pseudohyphal growth [Source:SGD;Acc:S000001053] 0.6617173812683 84 YBR044C Protein involved in assembly of the succinate dehydrogenase complex; mitochondrial; putative chaperone [Source:SGD;Acc:S000000248] 0.6608896282746369 85 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] 0.6603520376444654 87 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] 0.6596339998738501 88 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] 0.6592517237979897 89 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] 0.6583741754869215 90 YDR194C Mitochondrial transcription elongation factor; DEAD-box protein; required for efficient splicing of mitochondrial Group I and II introns; non-polar RNA helicase that also facilities strand annealing; promotes RNA folding by stabilizing an early assembly intermediate [Source:SGD;Acc:S000002602] 0.6566149499091944 92 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] 0.6566022644948972 93 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] 0.6564230384926925 94 YJL054W Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane [Source:SGD;Acc:S000003590] 0.6556899658711285 95 YDL202W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000002361] 0.6551763467583405 96 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] 0.6528799275383737 98 YDR322W Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000002730] 0.6523577430305002 99 YNL213C Protein of unknown function; null mutant lacks mitochondrial DNA and cannot grow on glycerol; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000005157] 0.6509044838130036 100