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NFU1 | 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). (256 aa) | ||||
DRE2 | Component of the cytosolic Fe-S protein assembly (CIA) machinery; contains an Fe-S cluster that receives electrons from NADPH via the action of Tah18p in an early step in the CIA pathway; ortholog of human Ciapin1; protein abundance increases in response to DNA replication stress; inviability of the null mutant is functionally complemented by human CIAPIN1. (348 aa) | ||||
SSQ1 | Heat shock protein SSQ1, mitochondrial; Mitochondrial hsp70-type molecular chaperone; required for assembly of iron/sulfur clusters into proteins at a step after cluster synthesis, and for maturation of Yfh1p, which is a homolog of human frataxin implicated in Friedreich's ataxia. (657 aa) | ||||
LIP5 | 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. (414 aa) | ||||
MGE1 | GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family. (228 aa) | ||||
GRX5 | Monothiol glutaredoxin-5, mitochondrial; Glutathione-dependent oxidoreductase; mitochondrial matrix protein involved at an early step in the biogenesis of iron-sulfur centers along with Bol1p; hydroperoxide and superoxide-radical responsive; monothiol glutaredoxin subfamily member along with Grx3p and Grx4p. (150 aa) | ||||
ISU1 | 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. (165 aa) | ||||
YAH1 | Adrenodoxin homolog, mitochondrial; Ferredoxin of the mitochondrial matrix; required for formation of cellular iron-sulfur proteins; involved in heme A biosynthesis; human homolog FDX1L can complement yeast by allowing growth during down-regulation of yeast YAH1; Belongs to the adrenodoxin/putidaredoxin family. (172 aa) | ||||
TAH18 | NADPH-dependent diflavin oxidoreductase 1; Conserved NAPDH-dependent diflavin reductase; component of an early step in the cytosolic Fe-S protein assembly (CIA) machinery; transfers electrons from NADPH to the Fe-S cluster of Dre2p; plays a pro-death role under oxidative stress; Tah18p-dependent nitric oxide synthesis confers high-temperature stress tolerance; possible target for development of antifungal drugs; In the N-terminal section; belongs to the flavodoxin family. (623 aa) | ||||
NFS1 | 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. (497 aa) | ||||
YFH1 | 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. (174 aa) | ||||
RLI1 | Translation initiation factor RLI1; Essential Fe-S protein; required for ribosome biogenesis, translation initiation/termination; facilitates binding of multifactor complex (MFC) of initiation factors to small ribosomal subunit; Dom34-Hbs1 complex and Rli1p work in dissociating inactive ribosomes, thereby facilitating translation restart; forms complex with Lto1p and Yae1p; dependency on ROS-labile FeS clusters, activity in nuclear ribosomal-subunit export impaired by mild oxidative stress. (608 aa) | ||||
ISD11 | 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. (94 aa) | ||||
NBP35 | Cytosolic Fe-S cluster assembly factor NBP35; Essential cytoplasmic iron-sulfur cluster binding protein; forms a complex with Cfd1p that is involved in iron-sulfur protein assembly in the cytosol; similar to P-loop NTPases. (328 aa) | ||||
CFD1 | Cytosolic Fe-S cluster assembly factor CFD1; Highly conserved iron-sulfur cluster binding protein; localized in the cytoplasm; forms a complex with Nbp35p that is involved in iron-sulfur protein assembly in the cytosol. (293 aa) | ||||
MET18 | DNA repair/transcription protein MET18/MMS19; Component of cytosolic iron-sulfur protein assembly (CIA) machinery; acts at a late step of Fe-S cluster assembly; forms the CIA targeting complex with Cia1p and Cia2p that directs Fe-S cluster incorporation into a subset of proteins involved in methionine biosynthesis, DNA replication and repair, transcription, and telomere maintenance; ortholog of human MMS19; Belongs to the MET18/MMS19 family. (1032 aa) | ||||
YAE1 | Protein that forms a complex with Lto1p and Rli1p; essential for growth under standard (aerobic) conditions but not under anaerobic conditions; may have a role in protection of ribosomal assembly and function from damage due to reactive oxygen species. (141 aa) | ||||
IBA57 | 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. (497 aa) |