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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) | ||||
JJJ3 | Diphthamide biosynthesis protein 4; Protein of unknown function; contains a CSL Zn finger and a DnaJ-domain; involved in diphthamide biosynthesis; ortholog human Dph4; Belongs to the DPH4 family. (172 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) | ||||
MET5 | Sulfite reductase beta subunit; involved in amino acid biosynthesis, transcription repressed by methionine; Belongs to the nitrite and sulfite reductase 4Fe-4S domain family. (1442 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) | ||||
PRI2 | Subunit of DNA primase; DNA primase is required for DNA synthesis and double-strand break repair; Belongs to the eukaryotic-type primase large subunit family. (528 aa) | ||||
SDH3 | Subunit of succinate dehydrogenase and of TIM22 translocase; functions as cytochrome b subunit of succinate dehydrogenase, which couples oxidation of succinate to transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; also required for mitochondrial inner membrane protein import as part of the TIM22 complex; SDH3 has a paralog, SHH3, that arose from the whole genome duplication. (198 aa) | ||||
DPH2 | 2-(3-amino-3-carboxypropyl)histidine synthase subunit 2; Protein required for synthesis of diphthamide; required along with Dph1p, Kti11p, Jjj3p, and Dph5p; diphthamide is a modified histidine residue of translation elongation factor 2 (Eft1p or Eft2p); may act in a complex with Dph1p and Kti11p. (534 aa) | ||||
ACP1 | Mitochondrial matrix acyl carrier protein; involved in biosynthesis of octanoate, which is a precursor to lipoic acid; activated by phosphopantetheinylation catalyzed by Ppt2p. (125 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) | ||||
MRS4 | Mitochondrial RNA-splicing protein MRS4; Iron transporter of the mitochondrial carrier family; mediates Fe2+ transport across the inner mitochondrial membrane; active under low-iron conditions; may transport other cations; protein abundance increases in response to DNA replication stress; MRS4 has a paralog, MRS3, that arose from the whole genome duplication. (304 aa) | ||||
CCP1 | Mitochondrial cytochrome-c peroxidase; degrades reactive oxygen species in mitochondria, involved in the response to oxidative stress. (361 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) | ||||
ISA1 | Iron-sulfur assembly protein 1; Protein required for maturation of mitochondrial [4Fe-4S] proteins; functions in a complex with Isa2p and possibly Iba57p; isa1 deletion causes loss of mitochondrial DNA and respiratory deficiency; depletion reduces growth on nonfermentable carbon sources; functional ortholog of bacterial A-type ISC proteins; human ISCA1 can complement isa1 null mutant. (250 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) | ||||
ERG3 | Delta(7)-sterol 5(6)-desaturase; C-5 sterol desaturase; glycoprotein that catalyzes the introduction of a C-5(6) double bond into episterol, a precursor in ergosterol biosynthesis; transcriptionally down-regulated when ergosterol is in excess; mutants are viable, but cannot grow on non-fermentable carbon sources; substrate of HRD ubiquitin ligase; mutation is functionally complemented by human SC5D. (365 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) | ||||
MSS51 | Protein MSS51, mitochondrial; Specific translational activator for the mitochondrial COX1 mRNA; loosely associated with the matrix face of the mitochondrial inner membrane; localizes to vacuole membrane in response to H2O2; influences both COX1 mRNA translation and Cox1p assembly into cytochrome c oxidase; binds to heme B, which may be a mechanism for sensing oxygen levels in order to regulate cytochrome c oxidase biogenesis. (436 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) | ||||
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) | ||||
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) | ||||
CYB2 | Cytochrome b2, mitochondrial; Cytochrome b2 (L-lactate cytochrome-c oxidoreductase); component of the mitochondrial intermembrane space, required for lactate utilization; expression is repressed by glucose and anaerobic conditions. (591 aa) | ||||
ERG5 | Cytochrome P450 61; C-22 sterol desaturase; a cytochrome P450 enzyme that catalyzes the formation of the C-22(23) double bond in the sterol side chain in ergosterol biosynthesis; may be a target of azole antifungal drugs. (538 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) | ||||
MMT1 | Putative metal transporter involved in mitochondrial iron accumulation; MMT1 has a paralog, MMT2, that arose from the whole genome duplication. (510 aa) | ||||
SCS7 | Ceramide very long chain fatty acid hydroxylase SCS7; Sphingolipid alpha-hydroxylase; functions in the alpha-hydroxylation of sphingolipid-associated very long chain fatty acids, has both cytochrome b5-like and hydroxylase/desaturase domains, not essential for growth; Belongs to the sterol desaturase family. SCS7 subfamily. (384 aa) | ||||
ADE4 | Amidophosphoribosyltransferase; Phosphoribosylpyrophosphate amidotransferase (PRPPAT); catalyzes first step of the 'de novo' purine nucleotide biosynthetic pathway; also known as amidophosphoribosyltransferase; In the C-terminal section; belongs to the purine/pyrimidine phosphoribosyltransferase family. (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) | ||||
FET4 | Low-affinity Fe(II) transporter of the plasma membrane; Belongs to the FET4 family. (552 aa) | ||||
POL1 | Catalytic subunit of the DNA polymerase I alpha-primase complex; required for the initiation of DNA replication during mitotic DNA synthesis and premeiotic DNA synthesis. (1468 aa) | ||||
CYB5 | Cytochrome b5; involved in the sterol and lipid biosynthesis pathways; acts as an electron donor to support sterol C5-6 desaturation. (120 aa) | ||||
URE2 | Nitrogen catabolite repression transcriptional regulator; inhibits GLN3 transcription in good nitrogen source; role in sequestering Gln3p and Gat1p to the cytoplasm; has glutathione peroxidase activity and can mutate to acquire GST activity; self-assembly under limited nitrogen conditions creates [URE3] prion and releases catabolite repression. (354 aa) | ||||
NAR1 | Cytosolic Fe-S cluster assembly factor NAR1; Subunit of the cytosolic iron-sulfur (FeS) protein assembly machinery; required for maturation of cytosolic and nuclear FeS proteins and for normal resistance to oxidative stress; deficiency results in shortened lifespan and sensitivity to paraquat; homologous to human Narf. (491 aa) | ||||
LTO1 | Essential protein that forms a complex with Rli1p and Yae1p; ortholog of human ORAOV1, which is overexpressed in solid tumors; inviability of null mutant under standard conditions is complemented by overexpression of ORAOV1; 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. (198 aa) | ||||
POL2 | Catalytic subunit of DNA polymerase (II) epsilon; a chromosomal DNA replication polymerase that exhibits processivity and proofreading exonuclease activity; participates in leading-strand synthesis during DNA replication; also involved in DNA synthesis during DNA repair; interacts extensively with Mrc1p. (2222 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) | ||||
NTG2 | Endonuclease III homolog 2; DNA N-glycosylase and apurinic/apyrimidinic (AP) lyase; involved in base excision repair, localizes to the nucleus; sumoylated; NTG2 has a paralog, NTG1, that arose from the whole genome duplication. (380 aa) | ||||
SMF1 | Manganese transporter SMF1; Divalent metal ion transporter; broad specificity for di-valent and tri-valent metals; post-translationally regulated by levels of metal ions; member of the Nramp family of metal transport proteins; Belongs to the NRAMP family. (575 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) | ||||
CYC2 | Mitochondrial peripheral inner membrane protein; contains a FAD cofactor in a domain exposed in the intermembrane space; exhibits redox activity in vitro; likely participates in ligation of heme to acytochromes c and c1 (Cyc1p and Cyt1p). (366 aa) | ||||
CYT1 | Cytochrome c1, heme protein, mitochondrial; Cytochrome c1; component of the mitochondrial respiratory chain; expression is regulated by the heme-activated, glucose-repressed Hap2p/3p/4p/5p CCAAT-binding complex. (309 aa) | ||||
HEM15 | 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. (393 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) | ||||
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) | ||||
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) | ||||
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) | ||||
FIT2 | Facilitator of iron transport 2; Mannoprotein that is incorporated into the cell wall; incorporated via a glycosylphosphatidylinositol (GPI) anchor; involved in the retention of siderophore-iron in the cell wall. (153 aa) | ||||
FIT3 | Facilitator of iron transport 3; Mannoprotein that is incorporated into the cell wall; incorporated via a glycosylphosphatidylinositol (GPI) anchor; involved in the retention of siderophore-iron in the cell wall. (204 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) | ||||
CHL1 | Probable DNA helicase; involved in sister-chromatid cohesion and genome integrity and interstrand cross-link repair; interacts with ECO1 and CTF18; mutants are defective in silencing, rDNA recombination, aging and the heat shock response; FANCJ-like helicase family member; mutations in the human homolog, DDX11/ChLR1, cause Warsaw breakage syndrome; Belongs to the DEAD box helicase family. DEAH subfamily. DDX11/CHL1 sub-subfamily. (861 aa) | ||||
ELP3 | Subunit of Elongator complex; Elongator is required for modification of wobble nucleosides in tRNA; exhibits histone acetyltransferase activity that is directed to histones H3 and H4; disruption confers resistance to K. lactis zymotoxin; human homolog ELP3 can partially complement yeast elp3 null mutant; Belongs to the ELP3 family. (557 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) | ||||
REV3 | Catalytic subunit of DNA polymerase zeta; involved in translesion synthesis during post-replication repair; required for mutagenesis induced by DNA damage; involved in double-strand break repair; forms a complex with Rev7p, Pol31p and Pol32p; Belongs to the DNA polymerase type-B family. (1504 aa) | ||||
COX10 | Protoheme IX farnesyltransferase, mitochondrial; Heme A:farnesyltransferase; catalyzes first step in conversion of protoheme to heme A prosthetic group required for cytochrome c oxidase activity; human ortholog COX10 can complement yeast cox10 null mutant; human ortholog COX10 is associated with mitochondrial disorders; Belongs to the UbiA prenyltransferase family. (462 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) | ||||
TYW1 | S-adenosyl-L-methionine-dependent tRNA 4-demethylwyosine synthase; Iron-sulfer protein required for synthesis of Wybutosine modified tRNA; Wybutosine is a modified guanosine found at the 3'-position adjacent to the anticodon of phenylalanine tRNA which supports reading frame maintenance by stabilizing codon-anticodon interactions; induction by Yap5p in response to iron provides protection from high iron toxicity; overexpression results in increased cellular iron; Belongs to the TYW1 family. (810 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) | ||||
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) | ||||
ROX1 | Heme-dependent repressor of hypoxic genes; mediates aerobic transcriptional repression of hypoxia induced genes such as COX5b and CYC7; repressor function regulated through decreased promoter occupancy in response to oxidative stress; contains an HMG domain that is responsible for DNA bending activity; involved in the hyperosmotic stress resistance. (368 aa) | ||||
ISA2 | Iron-sulfur assembly protein 2; Protein required for maturation of mitochondrial [4Fe-4S] proteins; functions in a complex with Isa1p and possibly Iba57p; localizes to the mitochondrial intermembrane space, overexpression of ISA2 suppresses grx5 mutations. (185 aa) | ||||
DAP1 | Damage response protein 1; Heme-binding protein; involved in regulation of cytochrome P450 protein Erg11p; damage response protein, related to mammalian membrane progesterone receptors; mutations lead to defects in telomeres, mitochondria, and sterol synthesis; Belongs to the cytochrome b5 family. MAPR subfamily. (152 aa) | ||||
COX1 | Subunit I of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits; Belongs to the heme-copper respiratory oxidase family. (534 aa) | ||||
BOL1 | BolA-like protein 1; Mitochondrial matrix protein involved in Fe-S cluster biogenesis; facilitates [4Fe-2S] cluster inception into mitochondrial proteins such as lipoate synthase and succinate dehydrogenase; interacts and may function with Grx5p at an early step in Fe-S cluster biosynthesis; forms dimeric complexes with Grx5p and Nfu1p that alter the stability of shared Fe/S clusters; sequence similarity to human BOLA family member, BOLA1 and S. pombe uvi31, a putative DNA repair protein. (110 aa) | ||||
BOL3 | BolA-like protein 3; Protein involved in Fe-S cluster transfer to mitochondrial clients; protects [4Fe-4S] clusters from damage due to oxidative stress by acting along with Nfu1p at a late step in the transfer of [4Fe-4S] clusters from the ISA complex to mitochondrial client proteins like lipoate synthase and succinate dehydrogenase; sequence similarity to human BOLA family member, BOLA3, mutations of which are associated with Multiple Mitochondria Dysfunctions Syndrome (MMDS2). (118 aa) | ||||
KTI11 | Diphthamide biosynthesis protein 3; Zn-ribbon protein that co-purifies with Dph1 and Dph2; in a complex required for synthesis of diphthamide on translation factor eEF2 and with Elongator subunits Iki3p, Elp2p, and Elp3p; involved in modification of wobble nucleosides in tRNAs; forms a stable heterodimer with Ats1p; Belongs to the DPH3 family. (82 aa) | ||||
GRX7 | Cis-golgi localized monothiol glutaredoxin; more similar in activity to dithiol than other monothiol glutaredoxins; involved in the oxidative stress response; does not bind metal ions; GRX7 has a paralog, GRX6, that arose from the whole genome duplication. (203 aa) | ||||
RIM2 | Mitochondrial carrier protein RIM2; Mitochondrial pyrimidine nucleotide transporter; imports pyrimidine nucleoside triphosphates and exports pyrimidine nucleoside monophosphates; member of the mitochondrial carrier family. (377 aa) | ||||
FTH1 | Putative high affinity iron transporter; involved in transport of intravacuolar stores of iron; forms complex with Fet5p; expression is regulated by iron; proposed to play indirect role in endocytosis; protein abundance increases in response to DNA replication stress. (465 aa) | ||||
MET8 | Siroheme biosynthesis protein MET8; Bifunctional dehydrogenase and ferrochelatase; involved in the biosynthesis of siroheme, a prosthetic group used by sulfite reductase; required for sulfate assimilation and methionine biosynthesis; Belongs to the precorrin-2 dehydrogenase / sirohydrochlorin ferrochelatase family. MET8 subfamily. (274 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) | ||||
GRX6 | Cis-golgi localized monothiol glutaredoxin, binds Fe-S cluster; more similar in activity to dithiol than other monothiol glutaredoxins; involved in the oxidative stress response; GRX6 has a paralog, GRX7, that arose from the whole genome duplication. (231 aa) | ||||
POL3 | Catalytic subunit of DNA polymerase delta; required for chromosomal DNA replication during mitosis and meiosis, intragenic recombination, repair of double strand DNA breaks, and DNA replication during nucleotide excision repair (NER). (1097 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) | ||||
GLT1 | NAD(+)-dependent glutamate synthase (GOGAT); synthesizes glutamate from glutamine and alpha-ketoglutarate; with Gln1p, forms the secondary pathway for glutamate biosynthesis from ammonia; expression regulated by nitrogen source; assembles into filaments as cells approach stationary phase and under cytosolic acidification and starvation conditions. (2145 aa) | ||||
DLD1 | Major mitochondrial D-lactate dehydrogenase; oxidizes D-lactate to pyruvate, transcription is heme-dependent, repressed by glucose, and derepressed in ethanol or lactate; located in the mitochondrial inner membrane. (587 aa) | ||||
DLD2 | D-2-hydroxyglutarate--pyruvate transhydrogenase DLD2; D-2-hydroxyglutarate dehydrogenase, and minor D-lactate dehydrogenase; mitochondrial matrix protein that oxidizes D-2-hydroxyglutarate (D-2HG), an oncometabolite, to alpha-ketoglutarate with a minor role in lactate catabolism; located in the mitochondrial matrix; Belongs to the FAD-binding oxidoreductase/transferase type 4 family. (530 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) | ||||
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) | ||||
SDH4 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Membrane anchor subunit of succinate dehydrogenase (SDH); involved in coupling the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; has similarity to human SDH subunit D (SDHD), which is implicated in paraganglioma. (181 aa) | ||||
CTA1 | Catalase A; breaks down hydrogen peroxide in the peroxisomal matrix formed by acyl-CoA oxidase (Pox1p) during fatty acid beta-oxidation; Belongs to the catalase family. (515 aa) | ||||
SUR2 | Sphingolipid C4-hydroxylase SUR2; Sphinganine C4-hydroxylase; catalyses the conversion of sphinganine to phytosphingosine in sphingolipid biosyntheis; Belongs to the sterol desaturase family. (349 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) | ||||
EFT2 | Elongation factor 2 (EF-2), also encoded by EFT1; catalyzes ribosomal translocation during protein synthesis; contains diphthamide, the unique posttranslationally modified histidine residue specifically ADP-ribosylated by diphtheria toxin; EFT2 has a paralog, EFT1, that arose from the whole genome duplication. (842 aa) | ||||
FIT1 | Facilitator of iron transport 1; Mannoprotein that is incorporated into the cell wall; incorporated via a glycosylphosphatidylinositol (GPI) anchor; involved in the retention of siderophore-iron in the cell wall. (528 aa) | ||||
CYC7 | Cytochrome c isoform 2, expressed under hypoxic conditions; also known as iso-2-cytochrome c; electron carrier of the mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; protein abundance increases in response to DNA replication stress; CYC7 has a paralog, CYC1, that arose from the whole genome duplication. (113 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) | ||||
DLD3 | D-2-hydroxyglutarate--pyruvate transhydrogenase DLD3; 2-hydroxyglutarate transhydrogenase, and minor D-lactate dehydrogenase; converts D-2-hydroxyglutarate (D-2HG), an oncometabolite, to alpha-ketoglutarate in the presence of FAD, with concomitant reduction of pyruvate to D-lactate; minor lactate dehydrogenase activity; component of the retrograde regulon that consists of genes whose expression are stimulated by damage to mitochondria and reduced in cells grown with glutamate as the sole nitrogen source; located in the cytoplasm. (496 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) | ||||
TPA1 | Prolyl 3,4-dihydroxylase TPA1; Fe(II)/2-oxoglutarate-dependent dioxygenase family member; catalyzes the repair of methyl-base lesions in both ss and dsDNA by oxidative demethylation; Poly(rA)-binding protein involved in mRNA poly(A) tail length and mRNA stability; role in translation termination efficiency; interacts with Sup45p (eRF1), Sup35p (eRF3) and Pab1p; similar to human prolyl 4-hydroxylase OGFOD1; binds Fe(II) and 2-oxoglutarate. (644 aa) | ||||
COX15 | Cytochrome c oxidase assembly protein COX15; Protein required for the hydroxylation of heme O to form heme A; heme A is an essential prosthetic group for cytochrome c oxidase; Belongs to the COX15/CtaA family. (486 aa) | ||||
FTR1 | High affinity iron permease; involved in the transport of iron across the plasma membrane; forms complex with Fet3p; expression is regulated by iron; protein abundance increases in response to DNA replication stress; Belongs to the oxidase-dependent Fe transporter (OFeT) (TC 9.A.10.1) family. (404 aa) | ||||
PAB1 | Polyadenylate-binding protein, cytoplasmic and nuclear; Poly(A) binding protein; part of the 3'-end RNA-processing complex, mediates interactions between the 5' cap structure and the 3' mRNA poly(A) tail, involved in control of poly(A) tail length, interacts with translation factor eIF-4G; stimulates, but is not required for the deadenylation activity of the Pan2p-Pan3p poly(A)-ribonuclease complex; Belongs to the polyadenylate-binding protein type-1 family. (577 aa) | ||||
RAD3 | 5' to 3' DNA helicase; involved in nucleotide excision repair and transcription; subunit of RNA polII initiation factor TFIIH and of Nucleotide Excision Repair Factor 3 (NEF3); homolog of human XPD protein; mutant has aneuploidy tolerance; protein abundance increases in response to DNA replication stress; Belongs to the helicase family. RAD3/XPD subfamily. (778 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) | ||||
LEU1 | 3-isopropylmalate dehydratase; Isopropylmalate isomerase; catalyzes the second step in the leucine biosynthesis pathway; Belongs to the aconitase/IPM isomerase family. (779 aa) | ||||
MPO1 | Uncharacterized endoplasmic reticulum membrane protein YGL010W; Protein involved in metabolism of phytosphingosine; not an essential gene. (174 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) | ||||
OLE1 | Acyl-CoA desaturase 1; Delta(9) fatty acid desaturase; required for monounsaturated fatty acid synthesis and for normal distribution of mitochondria. (510 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) | ||||
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) | ||||
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) | ||||
CTT1 | Cytosolic catalase T; has a role in protection from oxidative damage by hydrogen peroxide. (562 aa) | ||||
YHB1 | Flavohemoprotein; Nitric oxide oxidoreductase; flavohemoglobin that plays role in oxidative and nitrosative stress responses; protects against nitration of cellular targets and against cell growth inhibition under aerobic or anaerobic conditions; yeast flavohemoglobin Yhb1p and human homolog neuroglobin NGB protect cells against alpha-synuclein cytotoxicity and aggregate formation; protein increases in abundance, relocalizes from nucleus to cytoplasmic foci upon DNA replication stress; Belongs to the globin family. Two-domain flavohemoproteins subfamily. (399 aa) | ||||
COQ6 | Flavin-dependent monooxygenase involved in ubiquinone biosynthesis; responsible for hydroxylation at position C5 and deamination at C4 during ubiquinone (Coenzyme Q) biosynthesis; localizes to matrix face of mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; human homolog COQ6 can complement yeast null mutant and is implicated in steroid-resistant nephrotic syndrome (SRNS). (479 aa) | ||||
BIO2 | Biotin synthase, mitochondrial; Biotin synthase; catalyzes the conversion of dethiobiotin to biotin, which is the last step of the biotin biosynthesis pathway; complements E. coli bioB mutant; Belongs to the radical SAM superfamily. Biotin synthase family. (375 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) | ||||
ERG11 | Lanosterol 14-alpha-demethylase; catalyzes C-14 demethylation of lanosterol to form 4,4''-dimethyl cholesta-8,14,24-triene-3-beta-ol in ergosterol biosynthesis pathway; transcriptionally down-regulated when ergosterol is in excess; member of cytochrome P450 family; associated and coordinately regulated with the P450 reductase Ncp1p; human CYP51A1 functionally complements the lethality of the erg11 null mutation. (530 aa) | ||||
NCP1 | NADP-cytochrome P450 reductase; involved in ergosterol biosynthesis; associated and coordinately regulated with Erg11p; In the N-terminal section; belongs to the flavodoxin family. (691 aa) | ||||
CIA2 | MIP18 family protein YHR122W; 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 Met18p 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 FAM96B. (231 aa) | ||||
DNA2 | Tripartite DNA replication factor; single-stranded DNA-dependent ATPase, ATP-dependent nuclease, helicase; tracking protein for flap cleavage during Okazaki fragment maturation; involved in DNA repair/processing of meiotic DNA double strand breaks; component of telomeric chromatin with cell-cycle dependent localization; required for telomerase-dependent telomere synthesis; forms nuclear foci upon DNA replication stress; human homolog DNA2 complements yeast dna2 mutant. (1522 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) | ||||
CBR1 | NADH-cytochrome b5 reductase 1; Cytochrome b reductase; not essential for viability; also detected in mitochondria; mutation in conserved NADH binding domain of the human ortholog results in type I methemoglobinemia. (284 aa) | ||||
DPH1 | 2-(3-amino-3-carboxypropyl)histidine synthase subunit 1; Protein required for synthesis of diphthamide; required along with Dph2p, Kti11p, Jjj3p, and Dph5p; diphthamide is a modified histidine residue of translation elongation factor 2 (Eft1p or Eft2p); may act in a complex with Dph2p and Kti11p. (425 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) | ||||
YAP5 | Basic leucine zipper (bZIP) iron-sensing transcription factor; involved in diauxic shift; YAP5 has a paralog, YAP7, that arose from the whole genome duplication. (245 aa) | ||||
GTT1 | ER associated glutathione S-transferase; capable of homodimerization; glutathione transferase for Yvc1p vacuolar cation channel; expression induced during the diauxic shift and throughout stationary phase; functional overlap with Gtt2p, Grx1p, and Grx2p. (234 aa) | ||||
RNR2 | Ribonucleoside-diphosphate reductase small chain 1; Ribonucleotide-diphosphate reductase (RNR), small subunit; the RNR complex catalyzes the rate-limiting step in dNTP synthesis and is regulated by DNA replication and DNA damage checkpoint pathways via localization of the small subunits; RNR2 has a paralog, RNR4, that arose from the whole genome duplication; Belongs to the ribonucleoside diphosphate reductase small chain family. (399 aa) | ||||
MRS3 | Mitochondrial RNA-splicing protein MRS3; Iron transporter, mediates Fe2+ transport across inner mito membrane; mitochondrial carrier family member; active under low-iron conditions; may transport other cations; MRS3 has a paralog, MRS4, that arose from the whole genome duplication. (314 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) | ||||
BNA1 | 3-hydroxyanthranilate 3,4-dioxygenase; 3-hydroxyanthranilic acid dioxygenase; required for the de novo biosynthesis of NAD from tryptophan via kynurenine; expression regulated by Hst1p. (177 aa) | ||||
CYC1 | Cytochrome c, isoform 1; also known as iso-1-cytochrome c; electron carrier of mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; CYC1 has a paralog, CYC7, that arose from the whole genome duplication; human homolog CYC1 can complement yeast null mutant; mutations in human CYC1 cause insulin-responsive hyperglycemia. (109 aa) |