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.

Alcohol dehydrogenase isoenzyme type IV; dimeric enzyme demonstrated to be zinc-dependent despite sequence similarity to iron-activated alcohol dehydrogenases; transcription is induced in response to zinc deficiency.

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.

Iron-regulated transcriptional activator AFT1; Transcription factor involved in iron utilization and homeostasis; binds consensus site PyPuCACCCPu and activates transcription in response to changes in iron availability; in iron-replete conditions localization is regulated by Grx3p, Grx4p, and Fra2p, and promoter binding is negatively regulated via Grx3p-Grx4p binding; AFT1 has a paralog, AFT2, that arose from the whole genome duplication; relative distribution to the nucleus increases upon DNA replication stress.

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.

Iron-sulfur clusters transporter ATM1, mitochondrial; Mitochondrial inner membrane ATP-binding cassette (ABC) transporter; exports mitochondrially synthesized precursors of iron-sulfur (Fe/S) clusters to the cytosol; human homolog ABCB7 can complement yeast null mutant; Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Ferrochelatase; a mitochondrial inner membrane protein, catalyzes insertion of ferrous iron into protoporphyrin IX, the eighth and final step in the heme biosynthetic pathway; human homolog FECH can complement yeast mutant and allow growth of haploid null after sporulation of a heterozygous diploid.

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.

Conserved protein of the mitochondrial matrix; performs a scaffolding function during assembly of iron-sulfur clusters, interacts physically and functionally with yeast frataxin (Yfh1p); ISU1 has a paralog, ISU2, that arose from the whole genome duplication; isu1 isu2 double mutant is inviable; human homolog ISCU implicated in mitochondrial myopathy, can complement isu1 isu2 double mutant; Belongs to the NifU family.

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.

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.

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.

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.

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.

Mitochondrial phosphate carrier; imports inorganic phosphate into mitochondria; functionally redundant with Pic2p but more abundant than Pic2p under normal conditions; phosphorylated; Belongs to the mitochondrial carrier (TC 2.A.29) family.

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.

Frataxin homolog intermediate form; Mitochondrial matrix iron chaperone; oxidizes and stores iron; interacts with Isu1p to promote Fe-S cluster assembly; mutation results in multiple Fe/S-dependent enzyme deficiencies; human frataxin homolog FXN is mutated in Friedrich's ataxia; human FTL gene can complement yeast yfh1 null mutant.

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.

Glucose-6-phosphate dehydrogenase (G6PD); catalyzes the first step of the pentose phosphate pathway; involved in adapting to oxidative stress; protein abundance increases in response to DNA replication stress; homolog of human G6PD which is deficient in patients with hemolytic anemia; human G6PD can complement yeast zwf1 null mutant.

Alcohol dehydrogenase isoenzyme type IV; dimeric enzyme demonstrated to be zinc-dependent despite sequence similarity to iron-activated alcohol dehydrogenases; transcription is induced in response to zinc deficiency.

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.

Alcohol dehydrogenase isoenzyme type IV; dimeric enzyme demonstrated to be zinc-dependent despite sequence similarity to iron-activated alcohol dehydrogenases; transcription is induced in response to zinc deficiency.

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.

Alcohol dehydrogenase isoenzyme type IV; dimeric enzyme demonstrated to be zinc-dependent despite sequence similarity to iron-activated alcohol dehydrogenases; transcription is induced in response to zinc deficiency.

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.

Alcohol dehydrogenase isoenzyme type IV; dimeric enzyme demonstrated to be zinc-dependent despite sequence similarity to iron-activated alcohol dehydrogenases; transcription is induced in response to zinc deficiency.

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.

Alcohol dehydrogenase isoenzyme type IV; dimeric enzyme demonstrated to be zinc-dependent despite sequence similarity to iron-activated alcohol dehydrogenases; transcription is induced in response to zinc deficiency.

Zinc-regulated transporter 1; High-affinity zinc transporter of the plasma membrane; responsible for the majority of zinc uptake; transcription is induced under low-zinc conditions by the Zap1p transcription factor.

Alcohol dehydrogenase isoenzyme type IV; dimeric enzyme demonstrated to be zinc-dependent despite sequence similarity to iron-activated alcohol dehydrogenases; transcription is induced in response to zinc deficiency.

Glucose-6-phosphate dehydrogenase (G6PD); catalyzes the first step of the pentose phosphate pathway; involved in adapting to oxidative stress; protein abundance increases in response to DNA replication stress; homolog of human G6PD which is deficient in patients with hemolytic anemia; human G6PD can complement yeast zwf1 null mutant.