Sequence Description Alias PCC hrr 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] 0.7805454583182085 1 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] 0.7355855465460027 7 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] 0.7289861486902092 8 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] 0.7136230512235229 16 YGR255C Flavin-dependent monooxygenase involved in ubiquinone biosynthesis; responsible for hydroxylation at position C5 and deamination at C4 during ubiquinone (Coenzyme Q) biosynthesis; localizes to matrix face of mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; human homolog COQ6 can complement yeast null mutant and is implicated in steroid-resistant nephrotic syndrome (SRNS) [Source:SGD;Acc:S000003487] 0.7110139910924789 5 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] 0.6986755486504281 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] 0.696361793083155 46 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] 0.6951298045601351 49 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] 0.691726058974811 53 YBR044C Protein involved in assembly of the succinate dehydrogenase complex; mitochondrial; putative chaperone [Source:SGD;Acc:S000000248] 0.6842781203574916 35 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] 0.6838263501610483 69 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] 0.6819478144852614 15 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] 0.6818165265084086 69 YHR194W Mitochondrial protein that may have a role in phospholipid metabolism; inner membrane protein with similarity to Mdm32p; required for normal mitochondrial morphology and inheritance; interacts genetically with MMM1, MMM2, MDM10, MDM12, and MDM34 [Source:SGD;Acc:S000001237] 0.6808914299845489 14 YML057W Calcineurin A; one isoform (the other is Cna1p) of the catalytic subunit of calcineurin, a Ca++/calmodulin-regulated protein phosphatase which regulates Crz1p (a stress-response transcription factor), the other calcineurin subunit is CNB1; regulates the function of Aly1p alpha-arrestin; CMP2 has a paralog, CNA1, that arose from the whole genome duplication [Source:SGD;Acc:S000004521] 0.6723685834447313 17 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] 0.6625332854436161 96 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] 0.6616114859166483 63 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] 0.6603039314089358 82 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] 0.6598221407711042 42 YER078C Mitochondrial aminopeptidase; cleaves the N termini of at least 38 imported proteins after cleavage by the mitochondrial processing peptidase (MPP), thereby increasing their stability; member of the aminopeptidase P family [Source:SGD;Acc:S000000880] 0.6589552835179907 23 YOL096C O-methyltransferase; catalyzes two different O-methylation steps in ubiquinone (Coenzyme Q) biosynthesis; component of a mitochondrial ubiquinone-synthesizing complex; phosphoprotein [Source:SGD;Acc:S000005456] 0.6565664343858261 25 YHR009C Putative oxidoreductase involved in late endosome to Golgi transport; physical and genetical interactions with Btn2p; null mutant is viable, has extended S phase, and sensitive to expression of top1-T722A allele; similar to human FOXRED1 [Source:SGD;Acc:S000001051] 0.6560679039818184 26 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] 0.6558536888041699 86 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] 0.6553759487732103 28 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] 0.6532636261504453 99 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] 0.6523885106691723 63 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6392308044289591 59 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] 0.6381554885355152 36 YML110C 2-hexaprenyl-6-methoxy-1,4-benzoquinone methyltransferase; involved in ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; respiratory defect of the null mutant is partially complemented by human COQ5 [Source:SGD;Acc:S000004578] 0.6347152530823152 39 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] 0.6339161163335184 40 YHR028C Dipeptidyl aminopeptidase; synthesized as a glycosylated precursor; localizes to the vacuolar membrane; similar to Ste13p [Source:SGD;Acc:S000001070] 0.6321616228320428 69 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] 0.6274234576186001 48 YDR376W Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability [Source:SGD;Acc:S000002784] 0.6224830835466256 52 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] 0.6206426284503503 67 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] 0.6202091543900383 89 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] 0.6196872953549702 92 YMR261C Regulatory subunit of trehalose-6-phosphate synthase/phosphatase; involved in synthesis of storage carbohydrate trehalose; expression is induced by stress conditions and repressed by the Ras-cAMP pathway; TPS3 has a paralog, TSL1, that arose from the whole genome duplication [Source:SGD;Acc:S000004874] 0.6194611303537256 59 YGL084C Plasma membrane protein involved in remodeling GPI anchors; member of the MBOAT family of putative membrane-bound O-acyltransferases; role in misfolded protein quality control; proposed to be involved in glycerol transport; homolog of the mammalian Hedgehog pathway modulator HHATL; GUP1 has a paralog, GUP2, that arose from the whole genome duplication [Source:SGD;Acc:S000003052] 0.6167196354573989 97 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] 0.6163345588505662 87 YDL206W Putative protein of unknown function; YDL206W is not an essential protein [Source:SGD;Acc:S000002365] 0.6159602716009217 70 YKL140W Steryl ester hydrolase; one of three gene products (Yeh1p, Yeh2p, Tgl1p) responsible for steryl ester hydrolase activity and involved in sterol homeostasis; localized to lipid particle membranes [Source:SGD;Acc:S000001623] 0.6129943402708538 94 YFR045W Putative mitochondrial transport protein; null mutant is viable, exhibits decreased levels of chitin and normal resistance to calcofluor white [Source:SGD;Acc:S000001941] 0.6113590952656764 71 YJR065C Essential component of the Arp2/3 complex; Arp2/3 is a highly conserved actin nucleation center required for the motility and integrity of actin patches; involved in endocytosis and membrane growth and polarity [Source:SGD;Acc:S000003826] 0.6066299156260082 74 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] 0.6054926550821222 86 YNL011C Putative protein of unknown function; YNL011C is not an essential gene [Source:SGD;Acc:S000004956] 0.6052914750701983 76 YGR080W Twinfilin; highly conserved actin monomer-sequestering protein involved in regulation of the cortical actin cytoskeleton; coordinates actin filament severing and monomer sequestering at sites of rapid actin turnover; composed of two cofilin-like regions, stimulates actin depolymerization as does the mouse homolog, mTwf1 [Source:SGD;Acc:S000003312] 0.6027948761300116 81 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] 0.6018273096261131 82 YGL104C Mitochondrial protein; mutation affects vacuolar protein sorting; putative transporter; member of the sugar porter family; VPS73 has a paralog, YBR241C, that arose from the whole genome duplication [Source:SGD;Acc:S000003072] 0.5974593071155413 89 YHR086W RNA binding protein, component of the U1 snRNP protein; mutants are defective in meiotic recombination and in formation of viable spores, involved in the formation of DSBs through meiosis-specific splicing of REC107 pre-mRNA; Nam8p regulon embraces the meiotic pre-mRNAs of REC107, HFM1, AMA1 SPO22 and PCH2; the putative RNA binding domains RRM2 and RRM3 are required for Nam8p meiotic function [Source:SGD;Acc:S000001128] 0.5971008749552394 93 YOR155C Inosine 5'-monophosphate (IMP)-specific 5'-nucleotidase; catalyzes the breakdown of IMP to inosine; responsible for production of nicotinamide riboside and nicotinic acid riboside; expression positively regulated by nicotinic acid and glucose availability; does not show similarity to known 5'-nucleotidases from other organisms [Source:SGD;Acc:S000005681] 0.5970574675865985 94 YPL224C Putative metal transporter involved in mitochondrial iron accumulation; MMT2 has a paralog, MMT1, that arose from the whole genome duplication [Source:SGD;Acc:S000006145] 0.5958368184074903 96