Reactome: A Curated Pathway Database
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Details on Person UniProt:Q86U28 ISCA2

Class:IdReferenceGeneProduct:233072
_chainChangeLogtransit peptide:1-8 added on Fri February 6 2015;chain:9-154 added on Fri February 6 2015
_displayNameUniProt:Q86U28 ISCA2
_timestamp2025-08-15 22:01:13
chaintransit peptide:1-8
chain:9-154
checksumD6D16E4451F04625
commentFUNCTION 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.SUBUNIT Heterotetramer; forms a dimer of dimers with IBA57 (PubMed:31831856). Interacts with [2Fe-2S]-ISCA2 forming the heterodimer [2Fe- 2S]-ISCA2-IBA57 complex; [2Fe-2S] cluster binding is absolutely required to promote the complex formation (PubMed:30269484).INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the HesB/IscA family.SEQUENCE CAUTION Extended N-terminus.
created[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
descriptionrecommendedName: Iron-sulfur cluster assembly 2 homolog, mitochondrial alternativeName: HESB-like domain-containing protein 1
geneNameISCA2
HBLD1
identifierQ86U28
isSequenceChangedFALSE
keywordAlternative splicing
Disease variant
Iron
Iron-sulfur
Metal-binding
Mitochondrion
Primary mitochondrial disease
Proteomics identification
Reference proteome
Transit peptide
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameISCA2
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998373] ENSEMBL:ENSG00000165898 ISCA2 [Homo sapiens]
secondaryIdentifierISCA2_HUMAN
A6NFF1
A8K3W3
G3V291
Q8IYZ0
Q96BB2
sequenceLength154
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8971974] UniProt:Q86U28-1 ISCA2 [Homo sapiens]
[ReferenceIsoform:8971975] UniProt:Q86U28-2 ISCA2 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:5691207] ISCA2 [mitochondrial matrix] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q86U28 ISCA2 (233072)