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GDH2 | NAD(+)-dependent glutamate dehydrogenase; degrades glutamate to ammonia and alpha-ketoglutarate; expression sensitive to nitrogen catabolite repression and intracellular ammonia levels; genetically interacts with GDH3 by suppressing stress-induced apoptosis. (1092 aa) | ||||
COX11 | Cytochrome c oxidase assembly protein COX11, mitochondrial; 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; Belongs to the COX11/CtaG family. (300 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) | ||||
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) | ||||
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) | ||||
SCO1 | 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. (295 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) | ||||
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) | ||||
FAA1 | Long-chain-fatty-acid--CoA ligase 1; Long chain fatty acyl-CoA synthetase; activates fatty acids with a preference for C12:0-C16:0 chain lengths; role in the competitive import of long-chain fatty acids and sphingoid long-chain bases; accounts for most acyl-CoA synthetase activity; localizes to lipid particles and the plasma membrane; role in sphingolipid-to-glycerolipid metabolism; forms ER foci upon replication stress; faa1 faa4 double null complemented by any of human ACSBG1, ACSL1, 3, 4, 5, 6, SLC27A2, or 4. (700 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) | ||||
IDP3 | Peroxisomal NADP-dependent isocitrate dehydrogenase; catalyzes oxidation of isocitrate to alpha-ketoglutarate with the formation of NADP(H+), required for growth on unsaturated fatty acids; IDP3 has a paralog, IDP2, that arose from the whole genome duplication. (420 aa) | ||||
FAA4 | Long-chain-fatty-acid--CoA ligase 4; Long chain fatty acyl-CoA synthetase; activates fatty acids with a preference for C12:0-C16:0 chain lengths; role in the competitive import of long-chain fatty acids and sphingoid long-chain bases; role in stationary phase survival; localizes to lipid particles and the plasma membrane; role in sphingolipid-to-glycerolipid metabolism; forms cytoplasmic foci upon replication stress; faa1 faa4 double null complemented by any of human ACSBG1, ACSL1, 3, 4, 5, 6, SLC27A2, or 4; Belongs to the ATP-dependent AMP-binding enzyme family. (694 aa) | ||||
IDP2 | Cytosolic NADP-specific isocitrate dehydrogenase; catalyzes oxidation of isocitrate to alpha-ketoglutarate; levels are elevated during growth on non-fermentable carbon sources and reduced during growth on glucose; IDP2 has a paralog, IDP3, that arose from the whole genome duplication. (412 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) | ||||
DNM1 | Dynamin-related protein DNM1; Dynamin-related GTPase involved in mitochondrial organization; required for mitochondrial fission and inheritance; self assembles on the cytoplasmic face of mitochondrial tubules at sites where division will occur; participates in endocytosis and regulates peroxisome fission along with Vps1p; mutants in the human ortholog DNM1L, which mediates mitochondrial fission, peroxisomal division, autophagy, and mitophagy, are associated with slowly progressive infantile encephalopathy. (757 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) | ||||
IDP1 | Mitochondrial NADP-specific isocitrate dehydrogenase; catalyzes the oxidation of isocitrate to alpha-ketoglutarate; not required for mitochondrial respiration and may function to divert alpha-ketoglutarate to biosynthetic processes. (428 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) | ||||
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) | ||||
FAA3 | Long-chain-fatty-acid--CoA ligase 3; Long chain fatty acyl-CoA synthetase; activates imported fatty acids with a preference for C16:0-C18:0 chain lengths; green fluorescent protein (GFP)-fusion protein localizes to the cell periphery; Belongs to the ATP-dependent AMP-binding enzyme family. (694 aa) | ||||
COX15 | Cytochrome c oxidase assembly protein COX15; Protein required for the hydroxylation of heme O to form heme A; heme A is an essential prosthetic group for cytochrome c oxidase; Belongs to the COX15/CtaA family. (486 aa) | ||||
FAA2 | Long-chain-fatty-acid--CoA ligase 2; Medium chain fatty acyl-CoA synthetase; activates imported fatty acids; accepts a wide range of fatty acid chain lengths with a preference for medium chains, C9:0-C13:0; localized to the peroxisome; comparative analysis suggests that a mitochondrially targeted form may result from translation starting at a non-canonical codon upstream of the annotated start codon. (744 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) | ||||
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) |