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Metabolite; its structure appears as you zoom in

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Cofactors and co-substrates are written beside the reaction when you zoom in

Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway

Compartment transfer of the molecule it is linked to

Binding, release or complex event; its proteins are named in its details

Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

Transfer of Fe-S clusters to SDHB

FXN:NFS1:ISD11:ISCU:2Fe-2S Cluster + 2 ISCA1:ISCA2:4Fe-4S + SDHB + SDHAF1 + SDHAF3 → FXN:ISD11:NFS1:ISCU + 2 ISCA1:ISCA2 + Fe2+ + SDHB:2Fe-2S:3Fe-4S:4Fe-4S:SDHAF1:SDHAF3

Reactome: R-HSA-9854984
Compartment: mitochondrial matrix
Rhea: None recorded

Inputs

FXN:NFS1:ISD11:ISCU:2Fe-2S ClusterR-HSA-1362398, mitochondrial matrix
2 ISCA1:ISCA2:4Fe-4SR-HSA-8878796, mitochondrial matrix
SDHBR-HSA-9854983, mitochondrial matrix
SDHAF1R-HSA-9854988, mitochondrial matrix
SDHAF3R-HSA-9855015, mitochondrial matrix

Outputs

FXN:ISD11:NFS1:ISCUR-HSA-1362404, mitochondrial matrix
2 ISCA1:ISCA2R-HSA-8878840, mitochondrial matrix
Fe2+CHEBI:29033, Fe, mitochondrial matrix
SDHB:2Fe-2S:3Fe-4S:4Fe-4S:SDHAF1:SDHAF3R-HSA-9854993, mitochondrial matrix

Catalysed by

iron chaperone activity of 2 Iron:FXN [mitochondrial matrix]

Literature

Protein-mediated assembly of succinate dehydrogenase and its cofactors Crit Rev Biochem Mol Biol, 2015
A Single Adaptable Cochaperone-Scaffold Complex Delivers Nascent Iron-Sulfur Clusters to Mammalian Respiratory Chain Complexes I-III Cell Metab, 2017
Disease-Causing SDHAF1 Mutations Impair Transfer of Fe-S Clusters to SDHB Cell Metab, 2016
The LYR factors SDHAF1 and SDHAF3 mediate maturation of the iron-sulfur subunit of succinate dehydrogenase Cell Metab, 2014
Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes Essays Biochem, 2018