5mpo | pdb_00005mpo

Crystal structure of human molybdopterin synthase complex

spinqualitylabelsall models 5mpo, resolution 2.43Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 5mpo is a 4 chain structure with sequence from Human. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Gene: MOCS2, MOCO1 (HUMAN), MOCS2, MCBPE, MOCO1 (HUMAN) Activity: Molybdopterin synthase, with EC number 2.8.1.12 Resources: FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT Disease [MOC2A_HUMAN] Sulfite oxidase deficiency due to molybdenum cofactor deficiency type B. The disease is caused by mutations affecting the gene represented in this entry. [MOC2B_HUMAN] Molybdenum cofactor deficiency type B (MOCOD type B) [MIM:252150]: Autosomal recessive disease which leads to the pleiotropic loss of all molybdoenzyme activities and is characterized by severe neurological damage, neonatal seizures and early childhood death. Note=The disease is caused by mutations affecting the gene represented in this entry. Function [MOC2A_HUMAN] Acts as a sulfur carrier required for molybdopterin biosynthesis. Component of the molybdopterin synthase complex that catalyzes the conversion of precursor Z into molybdopterin by mediating the incorporation of 2 sulfur atoms into precursor Z to generate a dithiolene group. In the complex, serves as sulfur donor by being thiocarboxylated (-COSH) at its C-terminus by MOCS3. After interaction with MOCS2B, the sulfur is then transferred to precursor Z to form molybdopterin.[HAMAP-Rule:MF_03051] [MOC2B_HUMAN] Catalytic subunit of the molybdopterin synthase complex, a complex that catalyzes the conversion of precursor Z into molybdopterin. Acts by mediating the incorporation of 2 sulfur atoms from thiocarboxylated MOCS2A into precursor Z to generate a dithiolene group.

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