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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) | ||||
CCC2 | Cu(+2)-transporting P-type ATPase; required for export of copper from the cytosol into an extracytosolic compartment; similar to human proteins involved in Menkes and Wilsons diseases; protein abundance increases in response to DNA replication stress; affects TBSV model (+)RNA virus replication by regulating copper metabolism; human homologs ATP7A and ATP7B both complement yeast null mutant. (1004 aa) | ||||
CTH1 | mRNA decay factor CTH1; Member of the CCCH zinc finger family; similar to mammalian Tis11 protein, which activates transcription and also has a role in mRNA degradation; may function with Tis11p in iron homeostasis; CTH1 has a paralog, TIS11, that arose from the whole genome duplication. (325 aa) | ||||
GRX3 | Monothiol glutaredoxin-3; Glutathione-dependent oxidoreductase; hydroperoxide and superoxide-radical responsive; monothiol glutaredoxin subfamily member along with Grx4p and Grx5p; protects cells from oxidative damage; with Grx4p, binds to Aft1p in iron-replete conditions, promoting its dissociation from promoters; evidence exists indicating that the translation start site is not Met1 as currently annotated, but rather Met36; GRX3 has a paralog, GRX4, that arose from the whole genome duplication. (250 aa) | ||||
GGC1 | Mitochondrial GTP/GDP carrier protein 1; Mitochondrial GTP/GDP transporter; essential for mitochondrial genome maintenance; has a role in mitochondrial iron transport; member of the mitochondrial carrier family. (300 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) | ||||
GEX1 | Glutathione exchanger 1; Proton:glutathione antiporter; localized to the vacuolar and plasma membranes; imports glutathione from the vacuole and exports it through the plasma membrane; has a role in resistance to oxidative stress and modulation of the PKA pathway; GEX1 has a paralog, GEX2, that arose from a segmental duplication. (615 aa) | ||||
ARH1 | Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family. (493 aa) | ||||
GMC1 | Putative multicopper oxidase GMC1; Protein involved in meiotic progression; mutants are delayed in meiotic nuclear division and are defective in synaptonemal complex assembly; possible membrane-localized protein; SWAT-GFP and mCherry fusion proteins localize to the endoplasmic reticulum and vacuole respectively. (608 aa) | ||||
VMA3 | V-type proton ATPase subunit c; Proteolipid subunit c of the V0 domain of vacuolar H(+)-ATPase; dicyclohexylcarbodiimide binding subunit; required for vacuolar acidification and important for copper and iron metal ion homeostasis; Belongs to the V-ATPase proteolipid subunit family. (160 aa) | ||||
SIT1 | Siderophore iron transporter 1; Ferrioxamine B transporter; member of the ARN family of transporters that specifically recognize siderophore-iron chelates; transcription is induced during iron deprivation and diauxic shift; potentially phosphorylated by Cdc28p; Belongs to the major facilitator superfamily. (628 aa) | ||||
GRX4 | Monothiol glutaredoxin-4; Glutathione-dependent oxidoreductase; hydroperoxide and superoxide-radical responsive; monothiol glutaredoxin subfamily member along with Grx3p and Grx5p; protects cells from oxidative damage; with Grx3p, binds to Aft1p in iron-replete conditions, promoting its dissociation from promoters; mutant has increased aneuploidy tolerance; transcription regulated by Yap5p; GRX4 has a paralog, GRX3, that arose from the whole genome duplication. (244 aa) | ||||
FET5 | Iron transport multicopper oxidase FET5; Multicopper oxidase; integral membrane protein with similarity to Fet3p; may have a role in iron transport. (622 aa) | ||||
JAC1 | J-type co-chaperone JAC1, mitochondrial; Specialized J-protein that functions in Fe-S cluster biogenesis; functions with Hsp70 in Fe-S cluster biogenesis in mitochondria; involved in iron metabolism; contains a J domain typical to J-type chaperones; localizes to the mitochondrial matrix. (184 aa) | ||||
AFT1 | 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. (690 aa) | ||||
CUP2 | Transcriptional activator protein CUP2; Copper-binding transcription factor; activates transcription of the metallothionein genes CUP1-1 and CUP1-2 in response to elevated copper concentrations; required for regulation of copper genes in response to DNA-damaging reagents; CUP2 has a paralog, HAA1, that arose from the whole genome duplication. (225 aa) | ||||
AIM14 | Probable metalloreductase AIM14; NADPH oxidase localized to the perinuclear ER; produces superoxide from NADPH; overexpression causes MCA1 dependent apoptosis; likely involved in superoxide-mediated regulation of the actin cytoskeleton; member of a conserved superfamily of NADPH oxidases (NOX enzymes); has similarity to iron/copper reductases (FRE1-8), particularly Fre8p; Belongs to the ferric reductase (FRE) family. AIM14 subfamily. (570 aa) | ||||
BOL2 | BolA-like protein 2; Cytosolic protein involved in repression of iron regulon transcription; forms an iron-independent complex with Fra1p, Grx3p, and Grx4p; null mutant fails to repress the iron regulon and is sensitive to nickel; sequence similarity to human BOLA family member, BOLA2; Belongs to the BolA/IbaG family. (120 aa) | ||||
ERV1 | Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation. (189 aa) | ||||
MTM1 | Mitochondrial protein of the mitochondrial carrier family; high affinity pyridoxal 5'-phosphate (PLP) transporter, important for delivery of PLP cofactor to mitochondrial enzymes; involved in mitochondrial iron homeostasis and in activating mitochondrial Sod2p by facilitating insertion of an essential manganese cofactor. (366 aa) | ||||
ARN1 | ARN family transporter for siderophore-iron chelates; responsible for uptake of iron bound to ferrirubin, ferrirhodin, and related siderophores; protein increases in abundance and relocalizes to the vacuole upon DNA replication stress; Belongs to the major facilitator superfamily. (627 aa) | ||||
ARN2 | Siderophore iron transporter ARN2; Transporter; member of the ARN family of transporters that specifically recognize siderophore-iron chelates; responsible for uptake of iron bound to the siderophore triacetylfusarinine C; Belongs to the major facilitator superfamily. (620 aa) | ||||
GEF1 | Anion/proton exchange transporter GEF1; Voltage-gated chloride channel; localized to the golgi, the endosomal system, and plasma membrane; involved in cation homeostasis; highly homologous to vertebrate voltage-gated chloride channels; modulates TBSV model (+) RNA virus replication by regulating copper metabolism. (779 aa) | ||||
UTR1 | ATP-NADH kinase; phosphorylates both NAD and NADH; active as a hexamer; enhances the activity of ferric reductase (Fre1p); UTR1 has a paralog, YEF1, that arose from the whole genome duplication. (530 aa) | ||||
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) | ||||
OCT1 | Mitochondrial intermediate peptidase; cleaves destabilizing N-terminal residues of a subset of proteins upon import, after their cleavage by mitochondrial processing peptidase (Mas1p-Mas2p); may contribute to mitochondrial iron homeostasis. (772 aa) | ||||
FRE2 | Ferric/cupric reductase transmembrane component 2; Ferric reductase and cupric reductase; reduces siderophore-bound iron and oxidized copper prior to uptake by transporters; expression induced by low iron levels but not by low copper levels; Belongs to the ferric reductase (FRE) family. (711 aa) | ||||
GEX2 | Glutathione exchanger 2; Proton:glutathione antiporter; localized to the vacuolar and plasma membranes; expressed at a very low level; potential role in resistance to oxidative stress and modulation of the PKA pathway; GEX2 has a paralog, GEX1, that arose from a segmental duplication. (615 aa) | ||||
FRE6 | Ferric reductase transmembrane component 6; Putative ferric reductase with similarity to Fre2p; expression induced by low iron levels. (712 aa) | ||||
SMF3 | Iron transporter SMF3; Putative divalent metal ion transporter involved in iron homeostasis; transcriptionally regulated by metal ions; member of the Nramp family of metal transport proteins; protein abundance increases in response to DNA replication stress. (473 aa) | ||||
FRE8 | Probable ferric reductase transmembrane component 8; Protein with sequence similarity to iron/copper reductases; involved in iron homeostasis; deletion mutant has iron deficiency/accumulation growth defects; expression increased in the absence of copper-responsive transcription factor Mac1p. (686 aa) | ||||
TIS11 | mRNA decay factor CTH2; mRNA-binding protein expressed during iron starvation; binds to a sequence element in the 3'-untranslated regions of specific mRNAs to mediate their degradation; involved in iron homeostasis; protein increases in abundance and relative distribution to the nucleus increases upon DNA replication stress; TIS11 has a paralog, CTH1, that arose from the whole genome duplication. (285 aa) | ||||
HMX1 | Heme-binding protein HMX1; ER localized heme oxygenase; involved in heme degradation during iron starvation and in the oxidative stress response; expression is regulated by AFT1 and oxidative stress; relocates to the perinuclear region in the presence of oxidants. (317 aa) | ||||
FRE1 | Ferric/cupric reductase transmembrane component 1; Ferric reductase and cupric reductase; reduces siderophore-bound iron and oxidized copper prior to uptake by transporters; expression induced by low copper and iron levels. (686 aa) | ||||
CCC1 | Protein CCC1; Vacuolar Fe2+/Mn2+ transporter; suppresses respiratory deficit of yfh1 mutants, which lack the ortholog of mammalian frataxin, by preventing mitochondrial iron accumulation; relative distribution to the vacuole decreases upon DNA replication stress. (322 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) | ||||
MAC1 | Metal-binding activator 1; Copper-sensing transcription factor; involved in regulation of genes required for high affinity copper transport; required for regulation of yeast copper genes in response to DNA-damaging agents; undergoes changes in redox state in response to changing levels of copper or MMS. (417 aa) | ||||
FET3 | 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. (636 aa) | ||||
ERG29 | Uncharacterized protein YMR134W; Protein of unknown function involved in ergosterol biosynthesis; conditional mutants produce less ergosterol, display impaired oxygen consumption, respiratory growth, mitochondrial iron utilization, and are more sensitive to oxidative stress; mutant bm-8 has a growth defect on iron-limited medium that is complemented by overexpression of Yfh1p; protein localizes to the cytoplasm, ER and nuclear envelope; highly conserved in ascomycetes. (237 aa) | ||||
MMT1 | Putative metal transporter involved in mitochondrial iron accumulation; MMT1 has a paralog, MMT2, that arose from the whole genome duplication. (510 aa) | ||||
ATM1 | 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. (690 aa) | ||||
ATX1 | Metal homeostasis factor ATX1; Cytosolic copper metallochaperone; transports copper to the secretory vesicle copper transporter Ccc2p for eventual insertion into Fet3p, which is a multicopper oxidase required for high-affinity iron uptake; human homolog ATOX1 can complement yeast atx1 mutant; overexpression of human ATOX1 suppresses lysine auxotrophy of the yeast sod1 null mutant, as does overexpression of yeast ATX1. (73 aa) | ||||
FRE4 | Ferric reductase transmembrane component 4; Ferric reductase; reduces a specific subset of siderophore-bound iron prior to uptake by transporters; expression induced by low iron levels. (719 aa) | ||||
FRE7 | Ferric/cupric reductase transmembrane component 7; Putative ferric reductase with similarity to Fre2p; expression induced by low copper levels. (620 aa) | ||||
ENB1 | Siderophore iron transporter ENB1; Endosomal ferric enterobactin transporter; expressed under conditions of iron deprivation; member of the major facilitator superfamily; expression is regulated by Rcs1p and affected by chloroquine treatment. (606 aa) | ||||
ISU2 | Mitochondrial protein required for iron-sulfur protein synthesis; performs scaffolding function during Fe/S cluster assembly; involved in Fe-S cluster assembly for both mitochondrial and cytosolic proteins; protein abundance increases under DNA replication stress; ISU2 has a paralog, ISU1, 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. (156 aa) | ||||
FRE3 | Ferric reductase transmembrane component 3; Ferric reductase; reduces siderophore-bound iron prior to uptake by transporters; expression induced by low iron levels; Belongs to the ferric reductase (FRE) family. (711 aa) | ||||
FRE5 | Ferric reductase transmembrane component 5; Putative ferric reductase with similarity to Fre2p; expression induced by low iron levels; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies. (694 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) | ||||
AFT2 | Iron-regulated transcriptional activator; activates genes involved in intracellular iron use and required for iron homeostasis and resistance to oxidative stress; AFT2 has a paralog, AFT1, that arose from the whole genome duplication. (416 aa) | ||||
MMT2 | Putative metal transporter involved in mitochondrial iron accumulation; MMT2 has a paralog, MMT1, that arose from the whole genome duplication; Belongs to the cation diffusion facilitator (CDF) transporter (TC 2.A.4) family. SLC30A subfamily. (484 aa) | ||||
HAA1 | Transcriptional activator involved in adaptation to weak acid stress; activates transcription of TPO2, YRO2, and other genes encoding membrane stress proteins; HAA1 has a paralog, CUP2, that arose from the whole genome duplication; relocalizes from cytoplasm to nucleus upon DNA replication stress. (694 aa) |