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Proteins/UNIPROT:Q5T440

IBA57

Protein·UNIPROT:Q5T440

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Mitochondrial protein involved in the maturation of mitochondrial [4Fe-4S]-proteins in the late stage of the iron-sulfur cluster assembly pathway. Operates in cooperation with ISCA2 in the maturation of [4Fe-4S] proteins..

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Involved in the maturation of mitochondrial 2Fe-2S proteins in the late stage of the iron-sulfur cluster assembly pathway..

Function from UniProtKB (Q5T440), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

3 rows

hereditary spastic paraplegia 74 has a germline basis in IBA57DiseaseGenCC HGNC:27302|MONDO:0014644Inferred only
mitochondrial disease has a germline basis in IBA57DiseaseGenCC HGNC:27302|MONDO:0044970Inferred only
multiple mitochondrial dysfunctions syndrome 3 has a germline basis in IBA57DiseaseGenCC HGNC:27302|MONDO:0014132Inferred only

Statements

2 statements

2 statements · 2 reference databases · Subject: IBA57 · Sources: Hetionet, Human-GEM

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UBC interacts with IBA57ProteinHetionet hetionet:interacts:200205-7316Inferred only
di-mu-sulfido-diiron(2+) is a substrate of IBA57CompoundHuman-GEM MAR20184Inferred only

Reactions

1 reaction

2ATP+2water+NADPH(4-)+2di-mu-sulfido-diiron(2+)2ADP+3proton+NADP(3-)+2phosphate(3-)+tetra-mu3-sulfido-tetrairon(2+)
Catalysed by FDXR & HSPA9 & IBA57 & FDX2 & GLRX5 & ISCA2 & HSCB & ISCA1

Other names

3 names

Resolves to: IBA57

Also called: C1orf69Iron-sulfur cluster assembly factor homologIron-sulfur cluster assembly factor IBA57, mitochondrial

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Balanced

GO:0005759

Human-GEM MAR20184CC BY 4.0Reference data