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SCO2 | Protein anchored to mitochondrial inner membrane; may have a redundant function with Sco1p in delivery of copper to cytochrome c oxidase; interacts with Cox2p; SCO2 has a paralog, SCO1, that arose from the whole genome duplication. (301 aa) | ||||
SCO1 | Copper-binding protein of mitochondrial inner membrane; required for cytochrome c oxidase activity and respiration; may function to deliver copper to cytochrome c oxidase; similar to thioredoxins; SCO1 has a paralog, SCO2, that arose from the whole genome duplication. (295 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) | ||||
ATP16 | Delta subunit of the central stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase 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. (160 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) | ||||
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) | ||||
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) | ||||
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) | ||||
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) | ||||
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) | ||||
REX2 | Oligoribonuclease, mitochondrial; 3'-5' RNA exonuclease; involved in 3'-end processing of U4 and U5 snRNAs, 5S and 5.8S rRNAs, and RNase P and RNase MRP RNA; localized to mitochondria and null suppresses escape of mtDNA to nucleus in yme1 yme2 mutants; RNase D exonuclease. (269 aa) | ||||
LIP2 | Lipoyl ligase; involved in the modification of mitochondrial enzymes by the attachment of lipoic acid groups; Belongs to the LipB family. (328 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) | ||||
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) | ||||
YDJ1 | 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. (409 aa) | ||||
HOL1 | Protein HOL1; Putative transporter in the major facilitator superfamily; member of the 12-spanner drug:H(+) antiporter DHA1 family; mutations in membrane-spanning domains permit cation and histidinol uptake. (586 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) | ||||
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) | ||||
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) | ||||
COX11 | Cytochrome c oxidase assembly protein COX11, mitochondrial; Protein required for delivery of copper to Cox1p; mitochondrial inner membrane protein; association with mitochondrial ribosomes suggests that copper delivery may occur during translation of Cox1p; Belongs to the COX11/CtaG family. (300 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) | ||||
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) |