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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Mitochondrial Fe–S assembly

Stepwise construction and transfer of iron–sulfur clusters to mitochondrial recipient proteins. 6 reactions, from Reactome R-HSA-1362409.

  1. FXN:NFS1:ISD11:ISCU assembles 2Fe-2S iron-sulfur cluster2 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)
  2. Frataxin binds ironFXN:ISD11:NFS1:ISCU + 4 Fe2+ → 2 Iron:FXN:NFS1:ISD11:ISCU
  3. Mitoferrin translocates iron from the mitochondrial intermembrane space to the mitochondrial matrixFe2+ → Fe2+
  4. FDXR transfers electrons to FDX1,2 (FDX1L)FDXR:FADH2 + 2 FDX1,FDX2 (oxidized) → FDXR:FAD + 2 H+ + 2 FDX1,FDX2 (reduced)
  5. NADPH transfers electrons to FDXRNADPH + FDXR:FAD + H+ → NADP+ + FDXR:FADH2
  6. Formation of 4Fe-4S cluster on ISCA1:ISCA22 GLRX5:2Fe-2S + ISCA1:ISCA2 → ISCA1:ISCA2:4Fe-4S + 2 GLRX5