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A reference for what the literature reports. Not medical advice.

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Proteins/UNIPROT:Q86U28

ISCA2

Protein·UNIPROT:Q86U28

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Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway. May be involved in the binding of an intermediate of Fe/S cluster assembly..

Function from UniProtKB (Q86U28), 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

2 rows

mitochondrial disease has a germline basis in ISCA2DiseaseGenCC HGNC:19857|MONDO:0044970Inferred only
multiple mitochondrial dysfunctions syndrome 4 has a germline basis in ISCA2DiseaseGenCC HGNC:19857|MONDO:0014611Inferred only

Statements

8 statements

8 statements · 3 reference databases · Subject: ISCA2 · Sources: Hetionet, Human-GEM, Reactome

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NTNG1 covaries with ISCA2ProteinHetionet hetionet:covaries:122961-22854Inferred only
ISCA2 participates in Aerobic respiration and respiratory electron transportPathwayReactome R-HSA-1428517Inferred only
ISCA2 participates in Citric acid cycle (TCA cycle)PathwayReactome R-HSA-71403Inferred only
ISCA2 participates in Maturation of TCA enzymes and regulation of TCA cyclePathwayReactome R-HSA-9854311Inferred only
ISCA2 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
ISCA2 participates in Mitochondrial iron-sulfur cluster biogenesisPathwayReactome R-HSA-1362409Inferred only
di-mu-sulfido-diiron(2+) is a substrate of ISCA2CompoundHuman-GEM MAR20184Inferred only
tetra-mu3-sulfido-tetrairon(2+) is a substrate of ISCA2CompoundHuman-GEM MAR06403Inferred only

Reactions

2 reactions

MAM00210m+3proton+NADPH(4-)+2S-adenosyl-L-methionine+tetra-mu3-sulfido-tetrairon(2+)MAM00208m+2MAM01098m+4iron(2+)+2hydrogen sulfide+2L-methionine+NADP(3-)
Catalysed by LIAS & FDX1 & FDXR & GCSH & ISCA1 or LIAS

Other names

3 names

Resolves to: ISCA2

Also called: HBLD1HESB-like domain-containing protein 1Iron-sulfur cluster assembly 2 homolog, mitochondrial

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&

or LIAS & FDX1 & FDXR & GCSH & NFU1

Reaction pageLeft to rightBalancedEC 2.8.1.8mitochondrial matrix

Human-GEM MAR06403CC BY 4.0Reference data

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

Reaction pageLeft to rightBalancedmitochondrial matrix

Human-GEM MAR20184CC BY 4.0Reference data