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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

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Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

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Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
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.

FXN:NFS1:ISD11:ISCU assembles 2Fe-2S iron-sulfur cluster

2 Iron:FXN:NFS1:ISD11:ISCU + 4 L-Cys + 4 FDX1,FDX2 (reduced) → FXN:NFS1:ISD11:ISCU:2Fe-2S Cluster + 4 L-Ala + 4 FDX1,FDX2 (oxidized)

Reactome: R-HSA-1362408
Compartment: mitochondrial matrix
Rhea: None recorded

Inputs

2 Iron:FXN:NFS1:ISD11:ISCUR-HSA-1362401, mitochondrial matrix
4 L-CysCHEBI:35235, C3H7NO2S, mitochondrial matrix
4 FDX1,FDX2 (reduced)R-HSA-2408372, mitochondrial matrix

Outputs

FXN:NFS1:ISD11:ISCU:2Fe-2S ClusterR-HSA-1362398, mitochondrial matrix
4 L-AlaCHEBI:57972, C3H7NO2, mitochondrial matrix
4 FDX1,FDX2 (oxidized)R-HSA-2408370, mitochondrial matrix

Catalysed by

cysteine desulfurase activity of 2 Iron:FXN:NFS1:ISD11:ISCU [mitochondrial matrix]EC 2.8.1.7

Literature

Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex Biochemistry, 2010
Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis Hum Mol Genet, 2009
Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones Hum Mol Genet, 2007
Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins J Am Chem Soc, 2003
Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis Biochemistry, 2012
Both human ferredoxins 1 and 2 and ferredoxin reductase are important for iron-sulfur cluster biogenesis Biochim. Biophys. Acta, 2012