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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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).

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.

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).

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.

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).

Dihydroxy-acid dehydratase, mitochondrial; Dihydroxyacid dehydratase; catalyzes third step in the common pathway leading to biosynthesis of branched-chain amino acids.

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).

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.

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).

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.

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).

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.

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).

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.

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).

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.

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.

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.

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).

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.

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.