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ILV6 | Acetolactate synthase small subunit, mitochondrial; Regulatory subunit of acetolactate synthase; acetolactate synthase catalyzes the first step of branched-chain amino acid biosynthesis; enhances activity of the Ilv2p catalytic subunit, localizes to mitochondria. (309 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) | ||||
CIT2 | Citrate synthase, peroxisomal isozyme involved in glyoxylate cycle; catalyzes condensation of acetyl coenzyme A and oxaloacetate to form citrate; expression is controlled by Rtg1p and Rtg2p transcription factors; SCF-Ucc1 regulates level of Cit2p to maintain citrate homeostasis; oxaloacetate-dependent positive feedback loop inhibits Cit2p ubiquitination; CIT2 has a paralog, CIT1, that arose from the whole genome duplication. (460 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) | ||||
LYS21 | Homocitrate synthase, mitochondrial; Homocitrate synthase isozyme; catalyzes the condensation of acetyl-CoA and alpha-ketoglutarate to form homocitrate, which is the first step in the lysine biosynthesis pathway; LYS21 has a paralog, LYS20, that arose from the whole genome duplication; Belongs to the alpha-IPM synthase/homocitrate synthase family. Homocitrate synthase LYS20/LYS21 subfamily. (440 aa) | ||||
LYS20 | Homocitrate synthase isozyme and functions in DNA repair; catalyzes the condensation of acetyl-CoA and alpha-ketoglutarate to form homocitrate, which is the first step in the lysine biosynthesis pathway; LYS20 has a paralog, LYS21, that arose from the whole genome duplication; Belongs to the alpha-IPM synthase/homocitrate synthase family. Homocitrate synthase LYS20/LYS21 subfamily. (428 aa) | ||||
ALT2 | Probable alanine aminotransferase; Catalytically inactive alanine transaminase; expression is repressed in the presence of alanine and repression is mediated by Nrg1p; ALT2 has a paralog, ALT1, that arose from the whole genome duplication; Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. Alanine aminotransferase subfamily. (507 aa) | ||||
HOM2 | Aspartic beta semi-aldehyde dehydrogenase; catalyzes the second step in the common pathway for methionine and threonine biosynthesis; expression regulated by Gcn4p and the general control of amino acid synthesis; Belongs to the aspartate-semialdehyde dehydrogenase family. (365 aa) | ||||
LYS4 | Homoaconitase, mitochondrial; Homoaconitase; catalyzes the conversion of homocitrate to homoisocitrate, which is a step in the lysine biosynthesis pathway. (693 aa) | ||||
HOM3 | Aspartokinase; Aspartate kinase (L-aspartate 4-P-transferase); cytoplasmic enzyme that catalyzes the first step in the common pathway for methionine and threonine biosynthesis; expression regulated by Gcn4p and the general control of amino acid synthesis; Belongs to the aspartokinase family. (527 aa) | ||||
ARG5,6 | Acetylglutamate kinase and N-acetyl-gamma-glutamyl-phosphate reductase; N-acetyl-L-glutamate kinase (NAGK) catalyzes the 2nd and N-acetyl-gamma-glutamyl-phosphate reductase (NAGSA), the 3rd step in arginine biosynthesis; synthesized as a precursor which is processed in the mitochondrion to yield mature NAGK and NAGSA; enzymes form a metabolon complex with Arg2p; NAGK C-terminal domain stabilizes the enzymes, slows catalysis and is involved in feed-back inhibition by arginine. (863 aa) | ||||
LEU1 | 3-isopropylmalate dehydratase; Isopropylmalate isomerase; catalyzes the second step in the leucine biosynthesis pathway; Belongs to the aconitase/IPM isomerase family. (779 aa) | ||||
ARO8 | Aromatic/aminoadipate aminotransferase 1; Aromatic aminotransferase I; expression is regulated by general control of amino acid biosynthesis. (500 aa) | ||||
BAT1 | Branched-chain-amino-acid aminotransferase, mitochondrial; Mitochondrial branched-chain amino acid (BCAA) aminotransferase; preferentially involved in BCAA biosynthesis; homolog of murine ECA39; highly expressed during logarithmic phase and repressed during stationary phase; BAT1 has a paralog, BAT2, that arose from the whole genome duplication; Belongs to the class-IV pyridoxal-phosphate-dependent aminotransferase family. (393 aa) | ||||
LYS12 | Homoisocitrate dehydrogenase, mitochondrial; Homo-isocitrate dehydrogenase; an NAD-linked mitochondrial enzyme required for the fourth step in the biosynthesis of lysine, in which homo-isocitrate is oxidatively decarboxylated to alpha-ketoadipate. (371 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) | ||||
ACO2 | Homocitrate dehydratase, mitochondrial; Putative mitochondrial aconitase isozyme; similarity to Aco1p, an aconitase required for the TCA cycle; expression induced during growth on glucose, by amino acid starvation via Gcn4p, and repressed on ethanol. (789 aa) | ||||
ILV3 | Dihydroxy-acid dehydratase, mitochondrial; Dihydroxyacid dehydratase; catalyzes third step in the common pathway leading to biosynthesis of branched-chain amino acids. (585 aa) | ||||
BAT2 | Branched-chain-amino-acid aminotransferase, cytosolic; Cytosolic branched-chain amino acid (BCAA) aminotransferase; preferentially involved in BCAA catabolism; homolog of murine ECA39; highly expressed during stationary phase and repressed during logarithmic phase; BAT2 has a paralog, BAT1, that arose from the whole genome duplication; Belongs to the class-IV pyridoxal-phosphate-dependent aminotransferase family. (376 aa) | ||||
AAT1 | Mitochondrial aspartate aminotransferase; catalyzes the conversion of oxaloacetate to aspartate in aspartate and asparagine biosynthesis. (451 aa) | ||||
AAT2 | Aspartate aminotransferase, cytoplasmic; Cytosolic aspartate aminotransferase involved in nitrogen metabolism; localizes to peroxisomes in oleate-grown cells; Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. (418 aa) | ||||
ALT1 | Probable alanine aminotransferase, mitochondrial; Alanine transaminase (glutamic pyruvic transaminase); involved in alanine biosynthesis and catabolism; TOR1-independent role in determining chronological lifespan; expression is induced in the presence of alanine; repression is mediated by Nrg1p; ALT1 has a paralog, ALT2, that arose from the whole genome duplication; Alt2p is catalytically inactive; Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. Alanine aminotransferase subfamily. (592 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) | ||||
ACO1 | Aconitate hydratase, mitochondrial; Aconitase; required for the tricarboxylic acid (TCA) cycle and also independently required for mitochondrial genome maintenance; component of the mitochondrial nucleoid; mutation leads to glutamate auxotrophy; human homolog ACO2 can complement yeast null mutant. (778 aa) | ||||
ILV5 | Ketol-acid reductoisomerase, mitochondrial; Acetohydroxyacid reductoisomerase and mtDNA binding protein; involved in branched-chain amino acid biosynthesis and maintenance of wild-type mitochondrial DNA; found in mitochondrial nucleoids. (395 aa) | ||||
ARG7 | Mitochondrial ornithine acetyltransferase; catalyzes the fifth step in arginine biosynthesis; also possesses acetylglutamate synthase activity, regenerates acetylglutamate while forming ornithine; Belongs to the ArgJ family. (441 aa) | ||||
ILV2 | Acetolactate synthase catalytic subunit, mitochondrial; Acetolactate synthase; catalyses the first common step in isoleucine and valine biosynthesis and is the target of several classes of inhibitors, localizes to the mitochondria; expression of the gene is under general amino acid control; Belongs to the TPP enzyme family. (687 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) | ||||
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) | ||||
LEU4 | Alpha-isopropylmalate synthase (2-isopropylmalate synthase); the main isozyme responsible for the first step in the leucine biosynthesis pathway; LEU4 has a paralog, LEU9, that arose from the whole genome duplication; Belongs to the alpha-IPM synthase/homocitrate synthase family. LeuA type 2 subfamily. (619 aa) | ||||
CIT1 | Citrate synthase, mitochondrial; Citrate synthase; catalyzes the condensation of acetyl coenzyme A and oxaloacetate to form citrate; the rate-limiting enzyme of the TCA cycle; nuclear encoded mitochondrial protein; CIT1 has a paralog, CIT2, that arose from the whole genome duplication. (479 aa) | ||||
ARG8 | Acetylornithine aminotransferase, mitochondrial; Acetylornithine aminotransferase; catalyzes the fourth step in the biosynthesis of the arginine precursor ornithine; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (423 aa) | ||||
LEU9 | Alpha-isopropylmalate synthase II (2-isopropylmalate synthase); catalyzes the first step in the leucine biosynthesis pathway; the minor isozyme, responsible for the residual alpha-IPMS activity detected in a leu4 null mutant; LEU9 has a paralog, LEU4, that arose from the whole genome duplication; Belongs to the alpha-IPM synthase/homocitrate synthase family. LeuA type 2 subfamily. (604 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) | ||||
CIT3 | Dual specificity mitochondrial citrate and methylcitrate synthase; catalyzes the condensation of acetyl-CoA and oxaloacetate to form citrate and that of propionyl-CoA and oxaloacetate to form 2-methylcitrate. (486 aa) |