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.

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.

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.

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.

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.

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.

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.

Mitochondrial protein import protein MAS5; Type I HSP40 co-chaperone; involved in regulation of HSP90 and HSP70 functions; acts as an adaptor that helps Rsp5p recognize cytosolic misfolded proteins for ubiquitination after heat shock; critical for determining cell size at Start as a function of growth rate; involved in protein translocation across membranes; member of the DnaJ family; chimeric protein in which human p58IPK J domain replaces yeast Ydj1p J domain can complement yeast ydj1 mutant.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Iron transport multicopper oxidase FET3; Ferro-O2-oxidoreductase; multicopper oxidase that oxidizes ferrous (Fe2+) to ferric iron (Fe3+) for subsequent cellular uptake by transmembrane permease Ftr1p; required for high-affinity iron uptake and involved in mediating resistance to copper ion toxicity, belongs to class of integral membrane multicopper oxidases; protein abundance increases in response to DNA replication stress.

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.

Iron transport multicopper oxidase FET3; Ferro-O2-oxidoreductase; multicopper oxidase that oxidizes ferrous (Fe2+) to ferric iron (Fe3+) for subsequent cellular uptake by transmembrane permease Ftr1p; required for high-affinity iron uptake and involved in mediating resistance to copper ion toxicity, belongs to class of integral membrane multicopper oxidases; protein abundance increases in response to DNA replication stress.

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.

Iron transport multicopper oxidase FET3; Ferro-O2-oxidoreductase; multicopper oxidase that oxidizes ferrous (Fe2+) to ferric iron (Fe3+) for subsequent cellular uptake by transmembrane permease Ftr1p; required for high-affinity iron uptake and involved in mediating resistance to copper ion toxicity, belongs to class of integral membrane multicopper oxidases; protein abundance increases in response to DNA replication stress.

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.

Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system.

Protein ISD11; Cysteine desulfurase (Nfs1p) activator; essential for the formation of the persulfide intermediate at the desulfurase active site during pyridoxal phosphate-dependent desulfuration of cysteine; required for mitochondrial iron-sulfur cluster biosynthesis; exclusive to eukaryotes, implicated as eukaryotic supplement to the bacterium-derived Fe-S cluster (ISC) assembly apparatus; involved in regulation of iron metabolism; member of the LYR protein family.

Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system.

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.

Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system.

Lipoyl synthase, mitochondrial; Protein involved in biosynthesis of the coenzyme lipoic acid; has similarity to E. coli lipoic acid synthase; Belongs to the radical SAM superfamily. Lipoyl synthase family.

Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system.

Cysteine desulfurase, mitochondrial; Cysteine desulfurase; involved in iron-sulfur cluster (Fe/S) biogenesis and in thio-modification of mitochondrial and cytoplasmic tRNAs; essential protein located predominantly in mitochondria.

Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system.

NifU-like protein, mitochondrial; Protein involved in Fe-S cluster transfer to mitochondrial clients; protects [4Fe-4S] clusters from damage due to oxidative stress; acts along with Bol3 at a late step in the transfer of [4Fe-4S] clusters from the ISA complex to client proteins; Fe-S loaded homodimer at steady state; similar to NifU, a bacterial protein required for Fe/S cluster maturation; ortholog of the human NFU1, mutations of which are associated with Multiple Mitochondria Dysfunctions Syndrome (MMDS1).

Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system.

Mitochondrial protein involved in assembly of succinate dehydrogenase; has a role in maturation of the Sdh2p subunit; member of the LYR protein family; mutations in human ortholog SDHAF1 are associated with infantile leukoencephalopathy.

Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system.

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.