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| | COX6 | Subunit VI of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; expression is regulated by oxygen levels. (148 aa) |
| | COX1 | Subunit I of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits; Belongs to the heme-copper respiratory oxidase family. (534 aa) |
| | AI5_ALPHA | Truncated non-functional cytochrome oxidase 1; Endonuclease I-SceIV; involved in intron mobility; encoded by a mobile group I intron within the mitochondrial COX1 gene; In the N-terminal section; belongs to the heme-copper respiratory oxidase family. (630 aa) |
| | ATP8 | ATP synthase protein 8; Subunit 8 of the F0 sector of mitochondrial F1F0 ATP synthase; encoded on the mitochondrial genome; ATP8 and ATP6 mRNAs are not translated in the absence of the F1 sector of ATPase. (48 aa) |
| | ATP6 | Subunit a of the F0 sector of mitochondrial F1F0 ATP synthase; mitochondrially encoded; translation is specifically activated by Atp22p; ATP6 and ATP8 mRNAs are not translated in the absence of the F1 sector of ATPase; mutations in human ortholog MT-ATP6 are associated with neurodegenerative disorders such as Neurogenic Ataxia and Retinitis Pigmentosa (NARP), Leigh syndrome (LS), Charcot-Marie-Tooth (CMT), and ataxia telangiectasia. (259 aa) |
| | COB | Cytochrome b; mitochondrially encoded subunit of the ubiquinol-cytochrome c reductase complex which includes Cobp, Rip1p, Cyt1p, Cor1p, Qcr2p, Qcr6p, Qcr7p, Qcr8p, Qcr9p, and Qcr10p. (385 aa) |
| | OLI1 | F0-ATP synthase subunit c (ATPase-associated proteolipid); encoded on the mitochondrial genome; mutation confers oligomycin resistance; expression is specifically dependent on the nuclear genes AEP1 and AEP2. (76 aa) |
| | VAR1 | Mitochondrial ribosomal protein of the small subunit; mitochondrially-encoded; polymorphic in different strains due to variation in number of AAT (asparagine) codons; translated near the mitochondrial inner membrane; may have a role in loss of mitochondrial DNA under stress conditions. (398 aa) |
| | COX2 | Subunit II of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits. (251 aa) |
| | COX3 | Subunit III of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits. (269 aa) |
| | MDM10 | Mitochondrial distribution and morphology protein 10; Subunit of both the ERMES and the SAM complex; component of ERMES complex which acts as a molecular tether between the mitochondria and the ER, necessary for efficient phospholipid exchange between organelles and for mitophagy; SAM/TOB complex component that functions in the assembly of outer membrane beta-barrel proteins; involved in mitochondrial inheritance and morphology; ERMES complex is often co-localized with peroxisomes and concentrated areas of pyruvate dehydrogenase; Belongs to the MDM10 family. (493 aa) |
| | GEM1 | Outer mitochondrial membrane GTPase, subunit of the ERMES complex; potential regulatory subunit of the ERMES complex that links the ER to mitochondria and may promote inter-organellar calcium and phospholipid exchange as well as coordinating mitochondrial DNA replication and growth; cells lacking Gem1p contain collapsed, globular, or grape-like mitochondria; ortholog of metazoan Miro GTPases. (662 aa) |
| | MRPL16 | 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. (232 aa) |
| | COR1 | Core subunit of the ubiquinol-cytochrome c reductase complex; the ubiquinol-cytochrome c reductase complex (bc1 complex) is a component of the mitochondrial inner membrane electron transport chain. (457 aa) |
| | MRP21 | Mitochondrial ribosomal protein of the small subunit; MRP21 exhibits genetic interactions with mutations in the COX2 and COX3 mRNA 5'-untranslated leader sequences. (177 aa) |
| | ATP1 | Alpha subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated; N-terminally propionylated in vivo; Belongs to the ATPase alpha/beta chains family. (545 aa) |
| | ATP3 | Gamma subunit of the F1 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (311 aa) |
| | MIS1 | C-1-tetrahydrofolate synthase, mitochondrial; Mitochondrial C1-tetrahydrofolate synthase; involved in interconversion between different oxidation states of tetrahydrofolate (THF); provides activities of formyl-THF synthetase, methenyl-THF cyclohydrolase, and methylene-THF dehydrogenase. (975 aa) |
| | TIM12 | Mitochondrial import inner membrane translocase subunit TIM12; Essential protein of the inner mitochondrial membrane; peripherally localized; component of the TIM22 complex, which is a twin-pore translocase that mediates insertion of numerous multispanning inner membrane proteins. (109 aa) |
| | CBP6 | Cytochrome B pre-mRNA-processing protein 6; Mitochondrial protein required for translation of the COB mRNA; forms a complex with Cbp3p 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. (162 aa) |
| | MRPL36 | Mitochondrial ribosomal protein of the large subunit; overproduction suppresses mutations in the COX2 leader peptide-encoding region. (177 aa) |
| | MRPS9 | Mitochondrial ribosomal protein of the small subunit. (278 aa) |
| | NPL4 | Nuclear protein localization protein 4; Substrate-recruiting cofactor of the Cdc48p-Npl4p-Ufd1p segregase; assists Cdc48p in the dislocation of misfolded, polyubiquitinated ERAD substrates that are subsequently delivered to the proteasome for degradation; also involved in the regulated destruction of resident ER membrane proteins, such as HMG-CoA reductase (Hmg1/2p) and cytoplasmic proteins (Fbp1p); role in mobilizing membrane bound transcription factors by regulated ubiquitin/proteasome-dependent processing (RUP). (580 aa) |
| | PDB1 | E1 beta subunit of the pyruvate dehydrogenase (PDH) complex; PDH is an evolutionarily conserved multi-protein complex found in mitochondria. (366 aa) |
| | MRPS5 | Mitochondrial ribosomal protein of the small subunit. (307 aa) |
| | MIC12 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; forms a subcomplex with Mic10p and Mic27p whose assembly and stability requires cardiolipin. (106 aa) |
| | MRPL37 | Mitochondrial ribosomal protein of the large subunit. (105 aa) |
| | MRPL27 | Mitochondrial ribosomal protein of the large subunit; homolog of human Bcl-2 interacting protein BMRP. (146 aa) |
| | MGR1 | Subunit of the mitochondrial (mt) i-AAA protease supercomplex; i-AAA degrades misfolded mitochondrial proteins; forms a subcomplex with Mgr3p that binds to substrates to facilitate proteolysis; required for growth of cells lacking mtDNA. (417 aa) |
| | MIC10 | Conserved component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; forms a subcomplex with Mic12p and Mic27p whose assembly and stability requires cardiolipin; homo-oligomers cause membrane bending; ortholog of human MINOS1. (97 aa) |
| | MRPL32 | Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress. (183 aa) |
| | IMG1 | Mitochondrial ribosomal protein of the large subunit; required for respiration and for maintenance of the mitochondrial genome. (169 aa) |
| | IMG2 | Mitochondrial ribosomal protein of the large subunit; conserved in metazoa, with similarity to human mitochondrial ribosomal protein MRPL49. (146 aa) |
| | ATP16 | Delta subunit of the central stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated. (160 aa) |
| | MRP10 | Mitochondrial ribosomal protein of the small subunit; contains twin cysteine-x9-cysteine motifs; oxidized by Mia40p during import into mitochondria. (95 aa) |
| | COX9 | Subunit VIIa of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (59 aa) |
| | CDC48 | Cell division control protein 48; AAA ATPase; subunit of polyUb-selective segregase complex involved in ERAD, INM-associated degradation (INMAD), mitotic spindle disassembly, macroautophagy, PMN, ribosome-associated degradation, ribophagy, homotypic ER membrane fusion, SCF complex disassembly, cell wall integrity during heat stress, and telomerase regulation; mobilizes membrane-anchored transcription factors by regulated Ub/proteasome-dependent processing (RUP); human ortholog VCP complements a cdc48 mutant. (835 aa) |
| | MRPL11 | Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2. (249 aa) |
| | TIM22 | Mitochondrial import inner membrane translocase subunit TIM22; Essential core component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane; forms the channel through which proteins are imported; Belongs to the Tim17/Tim22/Tim23 family. (207 aa) |
| | EHD3 | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-CoA hydrolase; member of a family of enoyl-CoA hydratase/isomerases; non-tagged protein is detected in highly purified mitochondria in high-throughput studies; phosphorylated; mutation affects fluid-phase endocytosis. (500 aa) |
| | RSM10 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S10 ribosomal protein; essential for viability, unlike most other mitoribosomal proteins. (203 aa) |
| | VMS1 | Protein VMS1; Component of a Cdc48p-complex involved in protein quality control; exhibits cytosolic and ER-membrane localization, with Cdc48p, during normal growth, and contributes to ER-associated degradation (ERAD) of specific substrates at a step after their ubiquitination; forms a mitochondrially-associated complex with Cdc48p and Npl4p under oxidative stress that is required for ubiquitin-mediated mitochondria-associated protein degradation (MAD); conserved in C. elegans and humans; Belongs to the ANKZF1/VMS1 family. (632 aa) |
| | MRX14 | Putative mitochondrial ribosomal protein of the large subunit; similar to E. coli L34 ribosomal protein; required for respiratory growth, as are most mitochondrial ribosomal proteins; protein increases in abundance and relocalizes to the plasma membrane upon DNA replication stress. (105 aa) |
| | MRPL1 | Mitochondrial ribosomal protein of the large subunit. (285 aa) |
| | COX26 | Cytochrome c oxidase subunit 26, mitochondrial; Stabilizes or regulates formation of respiratory chain supercomplexes composed of Complex III (ubiquinol-cytochrome c reductase) and Complex IV (cytochrome c oxidase). (66 aa) |
| | KGD2 | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); Dihydrolipoyl transsuccinylase; component of the mitochondrial alpha-ketoglutarate dehydrogenase complex, which catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA in the TCA cycle; phosphorylated. (463 aa) |
| | RSM24 | Mitochondrial ribosomal protein of the small subunit. (319 aa) |
| | SDH4 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Membrane anchor subunit of succinate dehydrogenase (SDH); involved in coupling the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; has similarity to human SDH subunit D (SDHD), which is implicated in paraganglioma. (181 aa) |
| | MRPL7 | Mitochondrial ribosomal protein of the large subunit; MRPL7 produces both YmL5 and YmL7, which are two different modified forms of the same protein. (292 aa) |
| | MHR1 | 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. (226 aa) |
| | ATP5 | Subunit 5 of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; homologous to bovine subunit OSCP (oligomycin sensitivity-conferring protein); phosphorylated; Belongs to the ATPase delta chain family. (212 aa) |
| | CPR5 | Peptidyl-prolyl cis-trans isomerase (cyclophilin) of the ER; catalyzes the cis-trans isomerization of peptide bonds N-terminal to proline residues; transcriptionally induced in response to unfolded proteins in the ER; CPR5 has a paralog, CPR2, that arose from the whole genome duplication. (225 aa) |
| | TIM11 | Subunit e of mitochondrial F1F0-ATPase; ATPase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; essential for the dimeric and oligomeric state of ATP synthase, which in turn determines the shape of inner membrane cristae. (96 aa) |
| | MRPL35 | Mitochondrial ribosomal protein of the large subunit; Belongs to the phosphatidylethanolamine-binding protein family. Mitochondrion-specific ribosomal protein mL38 subfamily. (367 aa) |
| | MRPS28 | Mitochondrial ribosomal protein of the small subunit. (286 aa) |
| | MRP1 | 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. (321 aa) |
| | ATP17 | Subunit f of the F0 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (101 aa) |
| | MRP20 | Mitochondrial ribosomal protein of the large subunit. (263 aa) |
| | MRPL28 | Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress. (147 aa) |
| | RSM28 | Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation. (361 aa) |
| | QCR7 | Subunit 7 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; oriented facing the mitochondrial matrix; N-terminus appears to play a role in complex assembly; Belongs to the UQCRB/QCR7 family. (127 aa) |
| | TIM9 | Mitochondrial import inner membrane translocase subunit TIM9; Essential protein of the mitochondrial intermembrane space; forms a complex with Tim10p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane. (87 aa) |
| | RIP1 | Ubiquinol-cytochrome-c reductase; a Rieske iron-sulfur protein of the mitochondrial cytochrome bc1 complex; transfers electrons from ubiquinol to cytochrome c1 during respiration; during import, Rip1p is first imported into the mitochondrial matrix where it is processed, acquires its Fe-S cluster, and is folded, then is translocated into the inner membrane by the action of a homo-oligomer of Bcs1p, and finally is delivered by Bcs1p to Complex III for assembly. (215 aa) |
| | CYC7 | Cytochrome c isoform 2, expressed under hypoxic conditions; also known as iso-2-cytochrome c; electron carrier of the mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; protein abundance increases in response to DNA replication stress; CYC7 has a paralog, CYC1, that arose from the whole genome duplication. (113 aa) |
| | RML2 | 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. (393 aa) |
| | SOM1 | Protein SOM1, mitochondrial; Subunit of the mitochondrial inner membrane peptidase (IMP); IMP is required for maturation of mitochondrial proteins of the intermembrane space; Som1p facilitates cleavage of a subset of substrates; contains twin cysteine-x9-cysteine motifs. (74 aa) |
| | AFG3 | Mitochondrial respiratory chain complexes assembly protein AFG3; 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; In the N-terminal section; belongs to the AAA ATPase family. (761 aa) |
| | RSM18 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S18 ribosomal protein. (138 aa) |
| | PDA1 | E1 alpha subunit of the pyruvate dehydrogenase (PDH) complex; catalyzes the direct oxidative decarboxylation of pyruvate to acetyl-CoA; phosphorylated; regulated by glucose; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes. (420 aa) |
| | LPD1 | Dihydrolipoyl dehydrogenase, mitochondrial; Dihydrolipoamide dehydrogenase; the lipoamide dehydrogenase component (E3) of the pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase multi-enzyme complexes; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes; LPD1 has a paralog, IRC15, that arose from the whole genome duplication; Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. (499 aa) |
| | RPO41 | Mitochondrial RNA polymerase; single subunit enzyme similar to those of T3 and T7 bacteriophages; requires a specificity subunit encoded by MTF1 for promoter recognition; Mtf1p interacts with and stabilizes the Rpo41p-promoter complex, enhancing DNA bending and melting to facilitate pre-initiation open complex formation; Rpo41p also synthesizes RNA primers for mitochondrial DNA replication. (1351 aa) |
| | MIC19 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; Mic19p is peripheral to the inner membrane and may connect Mic60p with the Mic10p-Mic12p-Mic27p subcomplex; both yeast and human Mic19p become oxidized, and oxidation may regulate MICOS. (170 aa) |
| | QCR6 | Subunit 6 of the ubiquinol cytochrome-c reductase complex; the complex, also known as the cytochrome bc(1) complex or Complex III, is a component of the mitochondrial inner membrane electron transport chain; highly acidic protein; required for maturation of cytochrome c1; may be loosely associated with the complex since it is easily released into the intermembrane space; Belongs to the UQCRH/QCR6 family. (147 aa) |
| | YMR31 | 37S ribosomal protein YMR-31, mitochondrial; Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase; recruits E3 subunit (Lpd1p) to the E1-E2 (Kgd1p, Kgd2p) core; has similarity to human mitochondrial ribosomal protein MRP-S36. (123 aa) |
| | MNP1 | Mitochondrial ribosomal protein of the large subunit; has similarity to E. coli L7/L12 and human MRPL7 ribosomal proteins; associates with the mitochondrial nucleoid; required for normal respiratory growth. (194 aa) |
| | RSM23 | 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. (450 aa) |
| | COX4 | Subunit IV of cytochrome c oxidase; the terminal member of the mitochondrial inner membrane electron transport chain; precursor N-terminal 25 residues are cleaved during mitochondrial import; phosphorylated; spermidine enhances translation. (155 aa) |
| | COX13 | Subunit VIa of cytochrome c oxidase; present in a subclass of cytochrome c oxidase complexes that may have a role in mimimizing generation of reactive oxygen species; not essential for cytochrome c oxidase activity but may modulate activity in response to ATP; required for assembly of Rcf2p into cytochrome c oxidase - cytochrome bc1 supercomplexes. (129 aa) |
| | MDM34 | Mitochondrial distribution and morphology protein 34; Mitochondrial component of the ERMES complex; links the ER to mitochondria and may promote inter-organellar calcium and phospholipid exchange as well as coordinating mitochondrial DNA replication and growth; required for mitophagy; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase. (459 aa) |
| | TIM21 | Mitochondrial import inner membrane translocase subunit TIM21; Nonessential component of the TIM23 complex; interacts with the Translocase of the Outer Mitochondrial membrane (TOM complex) and with respiratory enzymes; may regulate the Translocase of the Inner Mitochondrial membrane (TIM23 complex) activity. (239 aa) |
| | KSS1 | Mitogen-activated protein kinase (MAPK); involved in signal transduction pathways that control filamentous growth and pheromone response; regulates septum assembly, and may directly phosphorylate Bni4p; the KSS1 gene is nonfunctional in S288C strains and functional in W303 strains. (368 aa) |
| | MRPL25 | Mitochondrial ribosomal protein of the large subunit; mutation confers increased replicative lifespan. (157 aa) |
| | TOM20 | Mitochondrial import receptor subunit TOM20; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; acts as a receptor for incoming precursor proteins. (183 aa) |
| | MRP13 | Mitochondrial ribosomal protein of the small subunit. (339 aa) |
| | PHB1 | Prohibitin-1; 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). (287 aa) |
| | MRPS35 | 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. (345 aa) |
| | TIM13 | Mitochondrial import inner membrane translocase subunit TIM13; Mitochondrial intermembrane space protein; forms a complex with Tim8p that delivers a subset of hydrophobic proteins to the TIM22 complex for insertion into the inner membrane. (105 aa) |
| | QCR9 | Subunit 9 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; required for electron transfer at the ubiquinol oxidase site of the complex; Belongs to the UQCR10/QCR9 family. (66 aa) |
| | PDX1 | E3-binding protein of the mitochondrial pyruvate dehydrogenase complex; plays a structural role in the complex by binding and positioning dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide acetyltransferase (E2) core. (410 aa) |
| | RSM27 | Mitochondrial ribosomal protein of the small subunit. (110 aa) |
| | MRPL9 | Mitochondrial ribosomal protein of the large subunit. (269 aa) |
| | PHB2 | Prohibitin-2; 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). (310 aa) |
| | MIC26 | MICOS subunit MIC26; Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; Mic26p is a non-essential component of the complex. (233 aa) |
| | MRP4 | Mitochondrial ribosomal protein of the small subunit. (394 aa) |
| | QCR10 | Subunit of the ubiqunol-cytochrome c oxidoreductase complex; this complex comprises part of the mitochondrial respiratory chain; members include Cobp, Rip1p, Cyt1p, Cor1p, Qcr2p, Qcr6p, Qcr7p, Qcr8p, Qcr9p, and Qcr10p and comprises part of the mitochondrial respiratory chain; Belongs to the UQCR11/QCR10 family. (77 aa) |
| | TIM10 | Mitochondrial import inner membrane translocase subunit TIM10; Essential protein of the mitochondrial intermembrane space; forms a complex with Tim9p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane. (93 aa) |
| | MAS2 | Alpha subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins. (482 aa) |
| | FYV4 | Protein FYV4, mitochondrial; Protein of unknown function; required for survival upon exposure to K1 killer toxin; Belongs to the mitochondrion-specific ribosomal protein mS41 family. (130 aa) |
| | PPE1 | Protein phosphatase methylesterase 1; Protein with carboxyl methyl esterase activity; may have a role in demethylation of the phosphoprotein phosphatase catalytic subunit; also identified as a small subunit mitochondrial ribosomal protein; Belongs to the AB hydrolase superfamily. (400 aa) |
| | SAM35 | Component of the sorting and assembly machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; the complex binds precursors of beta-barrel proteins and facilitates their insertion into the outer membrane. (329 aa) |
| | MRPL6 | Mitochondrial ribosomal protein of the large subunit. (214 aa) |
| | FMO1 | Flavin-containing monooxygenase; localized to the cytoplasmic face of the ER membrane; catalyzes oxidation of biological thiols to maintain the ER redox buffer ratio for correct folding of disulfide-bonded proteins. (432 aa) |
| | TIM44 | Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the TIM23 complex; tethers the import motor and regulatory factors (PAM complex) to the translocation channel (Tim23p-Tim17p core complex); TIM23 complex is short for the translocase of the inner mitochondrial membrane; Belongs to the Tim44 family. (431 aa) |
| | RSM25 | Mitochondrial ribosomal protein of the small subunit. (264 aa) |
| | COX5B | Subunit Vb of cytochrome c oxidase; cytochrome c oxidase is the terminal member of the mitochondrial inner membrane electron transport chain; Cox5Bp is predominantly expressed during anaerobic growth while its isoform Va (Cox5Ap) is expressed during aerobic growth; COX5B has a paralog, COX5A, that arose from the whole genome duplication. (150 aa) |
| | KGD1 | 2-oxoglutarate dehydrogenase, mitochondrial; Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase complex; catalyzes a key step in the tricarboxylic acid (TCA) cycle, the oxidative decarboxylation of alpha-ketoglutarate to form succinyl-CoA. (1014 aa) |
| | INA22 | Inner membrane assembly complex subunit 22; F1F0 ATP synthase peripheral stalk assembly factor; subunit of the matrix-exposed inner mitochondrial membrane localized INA complex (Ina22p-Ina17p) involved in assembly of the F1F0 peripheral stalk; co-purifies with Aim43p, ATP synthase subunits, and cytochrome bc1 complex assembly factors; interacts with Arh1p, a mitochondrial oxidoreductase; deletion mutant has a respiratory growth defect. (216 aa) |
| | TAD2 | Subunit of tRNA-specific adenosine-34 deaminase; forms a heterodimer with Tad3p that converts adenosine to inosine at the wobble position of several tRNAs; Belongs to the cytidine and deoxycytidylate deaminase family. ADAT2 subfamily. (250 aa) |
| | YJL045W | Succinate dehydrogenase [ubiquinone] flavoprotein subunit 2, mitochondrial; Minor succinate dehydrogenase isozyme; participates in oxidation of succinate and transfer of electrons to ubiquinone; induced during the diauxic shift in a Cat8p-dependent manner; YJL045W has a paralog, SDH1, that arose from the whole genome duplication. (634 aa) |
| | TIM54 | Mitochondrial import inner membrane translocase subunit TIM54; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane. (478 aa) |
| | MRPL8 | Mitochondrial ribosomal protein of the large subunit. (238 aa) |
| | ARG2 | Amino-acid acetyltransferase, mitochondrial; Acetylglutamate synthase (glutamate N-acetyltransferase); mitochondrial enzyme that catalyzes the first step in the biosynthesis of the arginine precursor ornithine; forms a complex with Arg5,6p. (574 aa) |
| | MRPL49 | Mitochondrial ribosomal protein of the large subunit. (161 aa) |
| | PAM16 | Subunit of the import motor (PAM complex); the PAM complex is a component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); forms a 1:1 subcomplex with Pam18p and inhibits its cochaperone activity; contains a J-like domain; Belongs to the TIM16/PAM16 family. (149 aa) |
| | TIM17 | Mitochondrial import inner membrane translocase subunit TIM17; Essential component of the TIM23 complex; with Tim23p, contributes to the architecture and function of the import channel; may link the import motor to the core Translocase of the Inner Mitochondrial membrane (TIM23 complex); Belongs to the Tim17/Tim22/Tim23 family. (158 aa) |
| | QCR8 | Subunit 8 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; oriented facing the intermembrane space; expression is regulated by Abf1p and Cpf1p; Belongs to the UQCRQ/QCR8 family. (94 aa) |
| | SSC1 | Heat shock protein SSC1, mitochondrial; 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. (654 aa) |
| | CYC1 | Cytochrome c, isoform 1; also known as iso-1-cytochrome c; electron carrier of mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; CYC1 has a paralog, CYC7, that arose from the whole genome duplication; human homolog CYC1 can complement yeast null mutant; mutations in human CYC1 cause insulin-responsive hyperglycemia. (109 aa) |
| | RSM26 | Mitochondrial ribosomal protein of the small subunit. (266 aa) |
| | RSM7 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S7 ribosomal protein. (247 aa) |
| | ATP2 | Beta subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated; Belongs to the ATPase alpha/beta chains family. (511 aa) |
| | TIM8 | Mitochondrial import inner membrane translocase subunit TIM8; Mitochondrial intermembrane space protein; forms a complex with Tim13p that delivers a subset of hydrophobic proteins to the TIM22 complex for inner membrane insertion; homolog of human TIMM8A, implicated in Mohr-Tranebjaerg syndrome, also known as deafness-dystonia-optic neuronopathy (DDON) syndrome; human TIMM8A can complement yeast null mutant. (87 aa) |
| | MRP17 | Mitochondrial ribosomal protein of the small subunit; MRP17 exhibits genetic interactions with PET122, encoding a COX3-specific translational activator. (131 aa) |
| | ATP7 | Subunit d of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (174 aa) |
| | YKL133C | Putative protein of unknown function; not required for growth of cells lacking the mitochondrial genome; SWAT-GFP and mCherry fusion proteins localize to the mitochondria; YKL133C has a paralog, MGR3, that arose from the whole genome duplication. (463 aa) |
| | OCT1 | Mitochondrial intermediate peptidase; cleaves destabilizing N-terminal residues of a subset of proteins upon import, after their cleavage by mitochondrial processing peptidase (Mas1p-Mas2p); may contribute to mitochondrial iron homeostasis. (772 aa) |
| | MRPL31 | Mitochondrial ribosomal protein of the large subunit. (131 aa) |
| | SDH3 | Subunit of succinate dehydrogenase and of TIM22 translocase; functions as cytochrome b subunit of succinate dehydrogenase, which couples oxidation of succinate to transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; also required for mitochondrial inner membrane protein import as part of the TIM22 complex; SDH3 has a paralog, SHH3, that arose from the whole genome duplication. (198 aa) |
| | SDH1 | Flavoprotein subunit of succinate dehydrogenase; couples the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; FAD binding to Sdh1p is required for the assembly of the succinate dehydrogenase subunits; mutations in human ortholog SDHA are associated with Leigh syndrome. (640 aa) |
| | RSM22 | Mitochondrial ribosomal protein of the small subunit; also predicted to be an S-adenosylmethionine-dependent RNA methyltransferase. (628 aa) |
| | MRP49 | Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation. (137 aa) |
| | MRPL38 | Mitochondrial ribosomal protein of the large subunit; appears as two protein spots (YmL34 and YmL38) on two-dimensional SDS gels; protein abundance increases in response to DNA replication stress. (138 aa) |
| | ACP1 | Mitochondrial matrix acyl carrier protein; involved in biosynthesis of octanoate, which is a precursor to lipoic acid; activated by phosphopantetheinylation catalyzed by Ppt2p. (125 aa) |
| | MRPL13 | Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation. (264 aa) |
| | MIC60 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; Mic60p is also involved in import of intermembrane space (IMS) proteins, probably by positioning Mia40p relative to the TOM complex to receive incoming proteins; ortholog of mammalian mitofilin. (540 aa) |
| | PAM17 | Presequence translocated-associated motor subunit PAM17, mitochondrial; Constituent of the TIM23 complex; proposed alternatively to be a component of the import motor (PAM complex) or to interact with and modulate the core TIM23 (Translocase of the Inner mitochondrial Membrane) complex; protein abundance increases in response to DNA replication stress; Belongs to the PAM17 family. (197 aa) |
| | TRZ1 | Ribonuclease Z; tRNA 3'-end processing endonuclease tRNase Z; also localized to mitochondria and interacts genetically with Rex2 exonuclease; homolog of the human candidate prostate cancer susceptibility gene ELAC2. (838 aa) |
| | MRPL20 | Mitochondrial ribosomal protein of the large subunit. (195 aa) |
| | OMA1 | Metalloendopeptidase of the mitochondrial inner membrane; important for adaptive responses to various homeostatic insults, preservation of normal mitochondrial function under damage-eliciting conditions, and stability of respiratory supercomplexes; involved in turnover of membrane-embedded proteins; mediates degradation of Cox1p in coa2 mutant cells; zebrafish ortholog has a role in organ development and mouse ortholog is also required for respiratory supercomplex stability. (345 aa) |
| | MMM1 | Maintenance of mitochondrial morphology protein 1; ER integral membrane protein, ERMES complex subunit; ERMES links the ER to mitochondria and may promote inter-organellar calcium and phospholipid exchange as well as coordinating mitochondrial DNA replication and growth; required for mitophagy; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase; Belongs to the MMM1 family. (426 aa) |
| | SDH2 | Iron-sulfur protein subunit of succinate dehydrogenase; the complex couples the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; other members are Sdh1p, Sdh3p, and Sdh4p. (266 aa) |
| | PAM18 | Subunit of the import motor (PAM complex); the PAM complex is a component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); essential J-protein cochaperone that stimulates Ssc1p ATPase activity to drive import; inhibited by Pam16p. (168 aa) |
| | COX12 | Subunit VIb of cytochrome c oxidase; cytochrome c oxidase is also known as respiratory Complex IV and is the terminal member of the mitochondrial inner membrane electron transport chain; required for assembly of cytochrome c oxidase but not required for activity after assembly; phosphorylated; easily released from the intermembrane space, suggesting a loose association with Complex IV. (83 aa) |
| | MAS1 | Beta subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins; Belongs to the peptidase M16 family. (462 aa) |
| | SHH4 | Mitochondrial inner membrane protein SHH4; Putative alternate subunit of succinate dehydrogenase (SDH); mitochondrial inner membrane protein; genetic interaction with SDH4 suggests that Shh4p can function as a functional SDH subunit; a fraction copurifies with SDH subunit Sdh3p; expression induced by nitrogen limitation in a GLN3, GAT1-dependent manner; Shh4p has greater similarity to human SDHD (subunit D of SDH, implicated in paraganglioma) than does its paralog Sdh4p; Belongs to the CybS family. (168 aa) |
| | QRI5 | Mitochondrial inner membrane protein; required for accumulation of spliced COX1 mRNA; may have an additional role in translation of COX1 mRNA. (111 aa) |
| | ATP14 | Subunit h of the F0 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; protein abundance increases in response to DNA replication stress. (124 aa) |
| | MRPL15 | Mitochondrial ribosomal protein of the large subunit; Belongs to the ribonuclease III family. Mitochondrion- specific ribosomal protein mL57 subfamily. (253 aa) |
| | TAD3 | Subunit of tRNA-specific adenosine-34 deaminase; forms a heterodimer with Tad2p that converts adenosine to inosine at the wobble position of several tRNAs; Belongs to the cytidine and deoxycytidylate deaminase family. ADAT3 subfamily. (322 aa) |
| | COX8 | Subunit VIII of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (78 aa) |
| | MRPL4 | Mitochondrial ribosomal protein of the large subunit; homolog of prokaryotic L29 ribosomal protein; located at the ribosomal tunnel exit. (319 aa) |
| | MRPL39 | Mitochondrial ribosomal protein of the large subunit. (70 aa) |
| | YML6 | 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. (286 aa) |
| | ATP18 | Subunit of the mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; termed subunit I or subunit j; does not correspond to known ATP synthase subunits in other organisms. (59 aa) |
| | RPM2 | Protein subunit of mitochondrial RNase P; has roles in nuclear transcription, cytoplasmic and mitochondrial RNA processing, and mitochondrial translation; distributed to mitochondria, cytoplasmic processing bodies, and the nucleus. (1202 aa) |
| | MRPL3 | 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; Belongs to the ribonuclease III family. Mitochondrion- specific ribosomal protein mL44 subfamily. (390 aa) |
| | IMP2-2 | Catalytic subunit of mitochondrial inner membrane peptidase complex; required for maturation of mitochondrial proteins of the intermembrane space; complex contains two catalytic subunits (Imp1p and Imp2p that differ in substrate specificity), and Som1p. (177 aa) |
| | SAM37 | Component of the Sorting and Assembly Machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; binds precursors of beta-barrel proteins and facilitates their outer membrane insertion; contributes to SAM complex stability. (327 aa) |
| | YTA12 | Mitochondrial respiratory chain complexes assembly protein YTA12; 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; In the N-terminal section; belongs to the AAA ATPase family. (825 aa) |
| | MGR3 | Subunit of the mitochondrial (mt) i-AAA protease supercomplex; i-AAA degrades misfolded mitochondrial proteins; forms a subcomplex with Mgr1p that binds to substrates to facilitate proteolysis; required for growth of cells lacking mtDNA. (501 aa) |
| | SHH3 | Putative mitochondrial inner membrane protein of unknown function; although similar to paralogous Sdh3p, Shh3p is not a stoichiometric subunit of either succinate dehydrogenase or of the TIM22 translocase; SHH3 has a paralog, SDH3, that arose from the whole genome duplication. (196 aa) |
| | IMP1 | Catalytic subunit of mitochondrial inner membrane peptidase complex; required for maturation of mitochondrial proteins of the intermembrane space; complex contains two catalytic subunits (Imp1p and Imp2p that differ in substrate specificty) and Som1p. (190 aa) |
| | MRPS8 | Mitochondrial ribosomal protein of the small subunit. (155 aa) |
| | MRPS17 | Mitochondrial ribosomal protein of the small subunit. (237 aa) |
| | MRPL24 | Mitochondrial ribosomal protein of the large subunit; two mitochondrial ribosomal proteins, YmL14 and YmL24, have been assigned to the same gene. (258 aa) |
| | TOM40 | Mitochondrial import receptor subunit TOM40; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; constitutes the core element of the protein conducting pore; pre-Tom40p is phosphorylated by PKA, which impairs its import into mitochondria under non-respiratory conditions. (387 aa) |
| | MRPL44 | Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress. (98 aa) |
| | MTF1 | Mitochondrial RNA polymerase specificity factor; has structural similarity to S-adenosylmethionine-dependent methyltransferases and functional similarity to bacterial sigma-factors; Mtf1p interacts with and stabilizes the Rpo41p-promoter complex, enhancing DNA bending and melting to facilitate pre-initiation open complex formation; Belongs to the class I-like SAM-binding methyltransferase superfamily. rRNA adenine N(6)-methyltransferase family. (341 aa) |
| | COX7 | Subunit VII of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (60 aa) |
| | MRPL33 | Mitochondrial ribosomal protein of the large subunit. (86 aa) |
| | DSS1 | 3'-5' exoribonuclease; component of the mitochondrial degradosome along with the ATP-dependent RNA helicase Suv3p; the degradosome associates with the ribosome and mediates turnover of aberrant or unprocessed RNAs. (969 aa) |
| | MRP7 | Mitochondrial ribosomal protein of the large subunit. (371 aa) |
| | SAM50 | Component of the Sorting and Assembly Machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; the complex binds precursors of beta-barrel proteins and facilitates their outer membrane insertion; homologous to bacterial Omp85. (484 aa) |
| | IDH1 | Subunit of mitochondrial NAD(+)-dependent isocitrate dehydrogenase; complex catalyzes the oxidation of isocitrate to alpha-ketoglutarate in the TCA cycle; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (360 aa) |
| | COX5A | Subunit Va of cytochrome c oxidase; cytochrome c oxidase is the terminal member of the mitochondrial inner membrane electron transport chain; Cox5Ap is predominantly expressed during aerobic growth while its isoform Vb (Cox5Bp) is expressed during anaerobic growth; COX5A has a paralog, COX5B, that arose from the whole genome duplication. (153 aa) |
| | TOM7 | Mitochondrial import receptor subunit TOM7; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; promotes assembly and stability of the TOM complex; Belongs to the Tom7 family. (60 aa) |
| | LAT1 | Dihydrolipoamide acetyltransferase component (E2) of the PDC; the pyruvate dehydrogenase complex (PDC) catalyzes the oxidative decarboxylation of pyruvate to acetyl-CoA. (482 aa) |
| | SWS2 | Putative mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S13 ribosomal protein; participates in controlling sporulation efficiency; localizes to vacuole in response to H2O2. (143 aa) |
| | MIC27 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; forms a subcomplex with Mic10p and Mic12p whose assembly and stability requires cardiolipin; Belongs to the apolipoprotein O/MICOS complex subunit Mic27 family. (234 aa) |
| | TOM70 | Mitochondrial import receptor subunit TOM70; Component of the TOM (translocase of outer membrane) complex; involved in the recognition and initial import steps for all mitochondrially directed proteins; acts as a receptor for incoming precursor proteins; TOM70 has a paralog, TOM71, that arose from the whole genome duplication. (617 aa) |
| | YNL122C | Mitochondrial ribosomal protein of the large subunit; homologous to bacterial L35 and human MRPL35 ribosomal proteins. (115 aa) |
| | TOM22 | Mitochondrial import receptor subunit TOM22; Component of the TOM (Translocase of Outer Membrane) complex; responsible for initial import of mitochondrially directed proteins; mediates interaction between TOM and TIM complexes and acts as a receptor for precursor proteins; Belongs to the Tom22 family. (152 aa) |
| | NAM9 | 37S ribosomal protein NAM9, mitochondrial; Mitochondrial ribosomal component of the small subunit; Belongs to the universal ribosomal protein uS4 family. (486 aa) |
| | MRPL22 | Mitochondrial ribosomal protein of the large subunit. (309 aa) |
| | MRPL19 | Mitochondrial ribosomal protein of the large subunit. (158 aa) |
| | ADE12 | Adenylosuccinate synthase; catalyzes the first step in synthesis of adenosine monophosphate from inosine 5'monophosphate during purine nucleotide biosynthesis; exhibits binding to single-stranded autonomously replicating (ARS) core sequence. (433 aa) |
| | MRPL17 | Mitochondrial ribosomal protein of the large subunit. (281 aa) |
| | MRPL10 | Mitochondrial ribosomal protein of the large subunit; appears as two protein spots (YmL10 and YmL18) on two-dimensional SDS gels. (322 aa) |
| | MRPS18 | Mitochondrial ribosomal protein of the small subunit; essential for viability, unlike most other mitoribosomal proteins. (217 aa) |
| | MDJ2 | Mitochondrial DnaJ homolog 2; Constituent of the mitochondrial import motor; associated with the presequence translocase; function overlaps with that of Pam18p; stimulates the ATPase activity of Ssc1p to drive mitochondrial import; contains a J domain. (146 aa) |
| | TIM23 | Essential component of the TIM23 complex; involved in protein import into mitochondrial matrix and inner membrane; with Tim17p, contributes to architecture and function of the import channel; TIM23 complex is short for the translocase of the inner mitochondrial membrane. (222 aa) |
| | MRPL50 | Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation. (139 aa) |
| | MRPS12 | 37S ribosomal protein S12, mitochondrial; Mitochondrial protein; may interact with ribosomes based on co-purification experiments; similar to E. coli and human mitochondrial S12 ribosomal proteins; Belongs to the universal ribosomal protein uS12 family. (153 aa) |
| | RSM19 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S19 ribosomal protein. (91 aa) |
| | MDM12 | Mitochondrial distribution and morphology protein 12; Mitochondrial outer membrane protein, ERMES complex subunit; required for transmission of mitochondria to daughter cells; required for mitophagy; may influence import and assembly of outer membrane beta-barrel proteins; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase; Belongs to the MDM12 family. (271 aa) |
| | ATP19 | Subunit k of the mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; associated only with the dimeric form of ATP synthase; Belongs to the ATP19 family. (68 aa) |
| | PTH4 | Putative uncharacterized protein YOL114C; Protein similar to the human peptidyl-tRNA hydrolase gene ICT1; associates with mitochondrial large subunit; may function in translation termination; YOL114C is not an essential gene. (202 aa) |
| | PET127 | Putative mitochondrial translation system component PET127; Protein with a role in 5'-end processing of mitochondrial RNAs; located in the mitochondrial membrane. (800 aa) |
| | TOM6 | Mitochondrial import receptor subunit TOM6; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; promotes assembly and stability of the TOM complex. (61 aa) |
| | CYT1 | Cytochrome c1, heme protein, mitochondrial; Cytochrome c1; component of the mitochondrial respiratory chain; expression is regulated by the heme-activated, glucose-repressed Hap2p/3p/4p/5p CCAAT-binding complex. (309 aa) |
| | IDH2 | Subunit of mitochondrial NAD(+)-dependent isocitrate dehydrogenase; complex catalyzes the oxidation of isocitrate to alpha-ketoglutarate in the TCA cycle; phosphorylated; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (369 aa) |
| | MRPL23 | Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2. (163 aa) |
| | PET123 | Mitochondrial ribosomal protein of the small subunit; PET123 exhibits genetic interactions with PET122, which encodes a COX3 mRNA-specific translational activator. (318 aa) |
| | MGE1 | GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family. (228 aa) |
| | TIM18 | Mitochondrial import inner membrane translocase subunit TIM18; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane; may mediate assembly or stability of the complex; Belongs to the CybS family. (192 aa) |
| | MIP1 | Mitochondrial DNA polymerase gamma; single subunit of mitochondrial DNA polymerase in yeast, in contrast to metazoan complex of catalytic and accessory subunits; polymorphic in yeast, petites occur more frequently in some lab strains; human ortholog POLG complements yeast mip1 mutant; mutations in human POLG associated with Alpers-Huttenlocher syndrome (AHS), progressive external ophthalmoplegia (PEO), parkinsonism, other mitochondrial diseases. (1254 aa) |
| | MRPS16 | Mitochondrial ribosomal protein of the small subunit. (121 aa) |
| | SUV3 | 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. (737 aa) |
| | TIM50 | Essential component of the TIM23 complex; acts as receptor for the translocase of the inner mitochondrial membrane (TIM23) complex guiding incoming precursors from the TOM complex; may control the gating of the Tim23p-Tim17p channel. (476 aa) |
| | ATP4 | Subunit b of the stator stalk of mitochondrial F1F0 ATP synthase; ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; contributes to the oligomerization of the complex, which in turn determines the shape of inner membrane cristae; phosphorylated; Belongs to the eukaryotic ATPase B chain family. (244 aa) |
| | MGR2 | Protein MGR2; Subunit of the TIM23 translocase complex; acts to couple Tim21p with the core Tim23 translocase; provides quality control for insertion of membrane proteins by regulating their release into the inner membrane by the TIM23 complex; absolutely required for mitochondrial import of presequence-containing proteins at elevated temperature; required for viability of cells lacking the mitochondrial genome (petite-negative phenotype); Belongs to the MGR2 family. (113 aa) |
| | INA17 | Inner membrane assembly complex subunit 17; F1F0 ATPase synthase peripheral stalk assembly factor; subunit of the matrix-exposed inner mitochondrial membrane localized INA complex (Ina22p-Ina17p) involved in assembly of the F1F0 peripheral stalk; co-purifies with Ina22p and ATP synthase subunits; null mutant displays elevated frequency of mitochondrial genome loss and has a respiratory growth defect. (182 aa) |
| | MRP51 | Mitochondrial ribosomal protein of the small subunit; MRP51 exhibits genetic interactions with mutations in the COX2 and COX3 mRNA 5'-untranslated leader sequences. (344 aa) |
| | MRPL40 | Mitochondrial ribosomal protein of the large subunit. (297 aa) |
| | RTC6 | 54S ribosomal protein RTC6, mitochondrial; Protein involved in translation; mutants have defects in biogenesis of nuclear ribosomes; sequence similar to prokaryotic ribosomal protein L36, may be a mitochondrial ribosomal protein; protein abundance increases in response to DNA replication stress; Belongs to the bacterial ribosomal protein bL36 family. (93 aa) |
| | CBP3 | 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. (335 aa) |
| | ATP15 | Epsilon subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated; Belongs to the eukaryotic ATPase epsilon family. (62 aa) |
| | ATP20 | Subunit g of the mitochondrial F1F0 ATP synthase; reversibly phosphorylated on two residues; unphosphorylated form is required for dimerization of the ATP synthase complex, which in turn determines oligomerization of the complex and the shape of inner membrane cristae. (115 aa) |
| | YME1 | Catalytic subunit of i-AAA protease complex; complex is located in mitochondrial inner membrane; responsible for degradation of unfolded or misfolded mitochondrial gene products; serves as nonconventional translocation motor to pull PNPase into intermembrane space; also has role in intermembrane space protein folding; mutation causes elevated rate of mitochondrial turnover; human homolog YME1L1 can complement yeast null mutant; In the C-terminal section; belongs to the peptidase M41 family. (747 aa) |
| | MRPL51 | Mitochondrial ribosomal protein of the large subunit. (140 aa) |
| | TOM5 | Mitochondrial import receptor subunit TOM5; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import of all mitochondrially directed proteins; involved in transfer of precursors from the Tom70p and Tom20p receptors to the Tom40p pore; Belongs to the Tom5 family. (50 aa) |
| | MRP2 | Mitochondrial ribosomal protein of the small subunit. (115 aa) |
| | QCR2 | Subunit 2 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; phosphorylated; transcription is regulated by Hap1p, Hap2p/Hap3p, and heme. (368 aa) |
