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Crystal structure of human Molybdopterin synthase catalytic subunit (MOCS2B)Crystal structure of human Molybdopterin synthase catalytic subunit (MOCS2B)
Structural highlights
DiseaseMOC2B_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. FunctionMOC2B_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.[1] [2] References
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