6wih | pdb_00006wih

N-terminal mutation of ISCU2 (L35H36) traps Nfs1 Cys loop in the active site of ISCU2 without metal present. Structure of human mitochondrial complex Nfs1-ISCU2(L35H36)-ISD11 with E.coli ACP1 at 1.9 A resolution (NIAU)2.

spinqualitylabelsall models 6wih, resolution 1.90Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 6wih is a 4 chain structure with sequence from Escherichia coli and Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Method: X-ray diffraction, Resolution 1.9Å Ligands: 1PE, 8Q1, EDO, EDT, GOL, P15, PEG, PG4, PGE, PLP Resources: FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT Disease NFS1_HUMAN Severe neonatal lactic acidosis due to NFS1-ISD11 complex deficiency. Function NFS1_HUMAN Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor. See Also Acyl carrier protein 3D structures Cysteine desulfurase 3D structures

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