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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) | ||||
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) | ||||
HEM25 | Mitochondrial glycine transporter; required for the transport of glycine into mitochondria for initiation of heme biosynthesis, with YMC1 acting as a secondary transporter; homolog of human SLC25A38, a mitochondrial glycine transporter associated with nonsyndromic autosomal recessive congenital sideroblastic anemia; human SLC25A38 can complement the heme deficiency associated with the null mutant; GFP-fusion protein is induced in response to the DNA-damaging agent MMS. (307 aa) | ||||
HEM12 | Uroporphyrinogen decarboxylase; catalyzes the fifth step in the heme biosynthetic pathway; localizes to both the cytoplasm and nucleus; a hem12 mutant has phenotypes similar to patients with porphyria cutanea tarda. (362 aa) | ||||
HEM1 | 5-aminolevulinate synthase, mitochondrial; 5-aminolevulinate synthase; catalyzes the first step in the heme biosynthetic pathway; an N-terminal signal sequence is required for localization to the mitochondrial matrix; expression is regulated by Hap2p-Hap3p; has a pyridoxal phosphate cofactor whose insertion is mediated by Mcx1p; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. (548 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) | ||||
HEM14 | Protoporphyrinogen oxidase; a mitochondrial enzyme that catalyzes the seventh step in the heme biosynthetic pathway, converting protoporphyrinogen IX to protoporphyrin IX; inhibited by diphenyl ether-type herbicides. (539 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) | ||||
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) | ||||
MTM1 | Mitochondrial protein of the mitochondrial carrier family; high affinity pyridoxal 5'-phosphate (PLP) transporter, important for delivery of PLP cofactor to mitochondrial enzymes; involved in mitochondrial iron homeostasis and in activating mitochondrial Sod2p by facilitating insertion of an essential manganese cofactor. (366 aa) | ||||
FLX1 | Mitochondrial FAD carrier protein FLX1; Mitochondrial flavin adenine dinucleotide transporter; FAD is a synthesis product of riboflavin; human homolog SLC25A32 is implicated in multiple acyl-CoA dehydrogenase deficiency (MADD) or glutaric aciduria type II (GAII), and can complement yeast null mutant; Belongs to the mitochondrial carrier (TC 2.A.29) family. (311 aa) | ||||
SFC1 | Mitochondrial succinate-fumarate transporter; transports succinate into and fumarate out of the mitochondrion; required for ethanol and acetate utilization. (322 aa) | ||||
NDI1 | Rotenone-insensitive NADH-ubiquinone oxidoreductase, mitochondrial; NADH:ubiquinone oxidoreductase; transfers electrons from NADH to ubiquinone in respiratory chain but does not pump protons, in contrast to higher eukaryotic multisubunit respiratory complex I; upon apoptotic stress, is activated in mitochondria by N-terminal cleavage, then translocates to cytoplasm to induce apoptosis; homolog of human AIFM2; yeast NDI1 complements several phenotypes of human cell line with mutated MT-ND4, implicated in Leber hereditary optic neuropathy. (513 aa) | ||||
PET8 | Putative mitochondrial carrier protein PET8; S-adenosylmethionine transporter of the mitochondrial inner membrane; member of the mitochondrial carrier family; required for biotin biosynthesis and respiratory growth. (284 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) | ||||
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) | ||||
ORT1 | Ornithine transporter of the mitochondrial inner membrane; exports ornithine from mitochondria as part of arginine biosynthesis; functionally complemented by human ortholog, SLC25A15, which is associated with hyperammonaemia-hyperornithinaemia-homocitrullinuria (HHH) syndrome, but HHH-associated variants fail to complement. (292 aa) | ||||
HEM4 | Uroporphyrinogen III synthase; catalyzes the conversion of hydroxymethylbilane to uroporphyrinogen III, the fourth step in heme biosynthesis; deficiency in the human homolog can result in the disease congenital erythropoietic porphyria. (275 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) |