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| MIA40 | 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. (403 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) | ||||
| 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) | ||||
| 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) | ||||
| 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) | ||||
| HIS3 | Imidazoleglycerol-phosphate dehydratase; catalyzes the sixth step in histidine biosynthesis; mutations cause histidine auxotrophy and sensitivity to Cu, Co, and Ni salts; transcription is regulated by general amino acid control via Gcn4p. (220 aa) | ||||
| ADE2 | Phosphoribosylaminoimidazole carboxylase; catalyzes a step in the 'de novo' purine nucleotide biosynthetic pathway; red pigment accumulates in mutant cells deprived of adenine. (571 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) | ||||
| MDH2 | Cytoplasmic malate dehydrogenase; one of three isozymes that catalyze interconversion of malate and oxaloacetate; involved in the glyoxylate cycle and gluconeogenesis during growth on two-carbon compounds; interacts with Pck1p and Fbp1. (377 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) | ||||
| 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) | ||||
| POR1 | Mitochondrial porin (voltage-dependent anion channel); outer membrane protein required for maintenance of mitochondrial osmotic stability and mitochondrial membrane permeability; couples the glutathione pools of the intermembrane space (IMS) and the cytosol; interacts with Om45 and Om14 in the outer membrane; phosphorylated; protein abundance increases in response to DNA replication stress. (283 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) | ||||
| 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) | ||||
| 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) | ||||
| RPS1B | Ribosomal protein 10 (rp10) of the small (40S) subunit; homologous to mammalian ribosomal protein S3A, no bacterial homolog; RPS1B has a paralog, RPS1A, that arose from the whole genome duplication. (255 aa) | ||||
| UBX2 | UBX domain-containing protein 2; Bridging factor involved in ER-associated protein degradation (ERAD); bridges the cytosolic Cdc48p-Npl1p-Ufd1p ATPase complex and the membrane associated Ssm4p and Hrd1p ubiquitin ligase complexes; contains a UBX (ubiquitin regulatory X) domain and a ubiquitin-associated (UBA) domain; redistributes from the ER to lipid droplets during the diauxic shift and stationary phase; required for the maintenance of lipid homeostasis. (584 aa) | ||||
| GLO1 | Lactoylglutathione lyase; Monomeric glyoxalase I; catalyzes the detoxification of methylglyoxal (a by-product of glycolysis) via condensation with glutathione to produce S-D-lactoylglutathione; expression regulated by methylglyoxal levels and osmotic stress. (326 aa) | ||||
| RPS1A | Ribosomal protein 10 (rp10) of the small (40S) subunit; homologous to mammalian ribosomal protein S3A, no bacterial homolog; RPS1A has a paralog, RPS1B, that arose from the whole genome duplication. (255 aa) | ||||
| FBP1 | Fructose-1,6-bisphosphatase; key regulatory enzyme in the gluconeogenesis pathway, required for glucose metabolism; undergoes either proteasome-mediated or autophagy-mediated degradation depending on growth conditions; glucose starvation results in redistribution to the periplasm; interacts with Vid30p; Belongs to the FBPase class 1 family. (348 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) | ||||
| 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) | ||||
| PCK1 | Phosphoenolpyruvate carboxykinase; key enzyme in gluconeogenesis, catalyzes early reaction in carbohydrate biosynthesis, glucose represses transcription and accelerates mRNA degradation, regulated by Mcm1p and Cat8p, located in the cytosol. (549 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) | ||||
| 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) | ||||
| PET9 | ADP,ATP carrier protein 2; Major ADP/ATP carrier of the mitochondrial inner membrane; exchanges cytosolic ADP for mitochondrially synthesized ATP; also imports heme and ATP; required for viability in many lab strains that carry a sal1 mutation; PET9 has a paralog, AAC3, that arose from the whole genome duplication; human homolog SLC25A4 implicated in progressive external ophthalmoplegia can complement yeast null mutant. (318 aa) | ||||
| GAL1 | Galactokinase; phosphorylates alpha-D-galactose to alpha-D-galactose-1-phosphate in the first step of galactose catabolism; expression regulated by Gal4p; human homolog GALK2 complements yeast null mutant; GAL1 has a paralog, GAL3, that arose from the whole genome duplication. (528 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) | ||||
| VID24 | Vacuolar import and degradation protein 24; GID Complex regulatory subunit; binds GID Complex in response to glucose through interactions with complex member Vid28p; regulates fructose-1,6-bisphosphatase (FBPase) targeting to the vacuole; promotes proteasome-dependent catabolite degradation of FBPase; peripheral membrane protein located at Vid (vacuole import and degradation) vesicles; Belongs to the GID4/VID24 family. (362 aa) | ||||
| LYS2 | Alpha aminoadipate reductase; catalyzes the reduction of alpha-aminoadipate to alpha-aminoadipate 6-semialdehyde, which is the fifth step in biosynthesis of lysine; activation requires posttranslational phosphopantetheinylation by Lys5p; Belongs to the ATP-dependent AMP-binding enzyme family. (1392 aa) | ||||
| LEU2 | Beta-isopropylmalate dehydrogenase (IMDH); catalyzes the third step in the leucine biosynthesis pathway; can additionally catalyze the conversion of beta-ethylmalate into alpha-ketovalerate; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (364 aa) | ||||
| RPN4 | Protein RPN4; Transcription factor that stimulates expression of proteasome genes; Rpn4p levels are in turn regulated by the 26S proteasome in a negative feedback control mechanism; RPN4 is transcriptionally regulated by various stress responses; relative distribution to the nucleus increases upon DNA replication stress. (531 aa) | ||||
| TRP1 | Phosphoribosylanthranilate isomerase; catalyzes the third step in tryptophan biosynthesis; in 2004, the sequence of TRP1 from strain S228C was updated by changing the previously annotated internal STOP (TAA) to serine (TCA); enhances vegetative growth at low and high temperatures when used as an auxotrophic marker in strains such as W303. (224 aa) | ||||
| UBC8 | Ubiquitin-conjugating enzyme that regulates gluconeogenesis; negatively regulates gluconeogenesis by mediating the glucose-induced ubiquitination of fructose-1,6-bisphosphatase (FBPase); cytoplasmic enzyme that catalyzes the ubiquitination of histones in vitro. (218 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) | ||||
| URA3 | Orotidine-5'-phosphate (OMP) decarboxylase; catalyzes the sixth enzymatic step in the de novo biosynthesis of pyrimidines, converting OMP into uridine monophosphate (UMP); converts 5-FOA into 5-fluorouracil, a toxic compound. (267 aa) | ||||
| ICL1 | Isocitrate lyase; catalyzes the formation of succinate and glyoxylate from isocitrate, a key reaction of the glyoxylate cycle; expression of ICL1 is induced by growth on ethanol and repressed by growth on glucose. (557 aa) | ||||
| OXA1 | Mitochondrial inner membrane insertase; mediates the insertion of both mitochondrial- and nuclear-encoded proteins from the matrix into the inner membrane; also has a role in insertion of carrier proteins into the inner membrane; acts as a voltage-gated ion channel, activated by substrate peptides; interacts with mitochondrial ribosomes; conserved from bacteria to animals. (402 aa) | ||||
| MDJ1 | DnaJ homolog 1, mitochondrial; Co-chaperone that stimulates HSP70 protein Ssc1p ATPase activity; involved in protein folding/refolding in the mitochodrial matrix; required for proteolysis of misfolded proteins; member of the HSP40 (DnaJ) family of chaperones. (511 aa) | ||||
| ERV1 | Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation. (189 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) | ||||
| YGR066C | Uncharacterized protein YGR066C; Putative protein of unknown function. (292 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) | ||||
| 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) | ||||
| ARG4 | Argininosuccinate lyase; catalyzes the final step in the arginine biosynthesis pathway; Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. (463 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) | ||||
| 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) | ||||
| TOM71 | Protein TOM71; Mitochondrial outer membrane protein; probable minor component of the TOM (translocase of outer membrane) complex responsible for recognition and import of mitochondrially directed proteins; TOM71 has a paralog, TOM70, that arose from the whole genome duplication. (639 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) | ||||
| 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) | ||||
| 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) | ||||
| SOD1 | Cytosolic copper-zinc superoxide dismutase; detoxifies superoxide; stabilizes Yck1p and Yck2p kinases in glucose to repress respiration; phosphorylated by Dun1p, enters nucleus under oxidative stress to promote transcription of stress response genes; human ortholog SOD1 implicated in ALS complements a null allele; abundance increases under DNA replication stress and during exposure to boric acid; localization to mitochondrial intermembrane space is modulated by MICOS complex; Belongs to the Cu-Zn superoxide dismutase family. (154 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) | ||||
| 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) | ||||