Structural highlights
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][1] [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.[2] [3] References
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This page was last modified 16:28, 2 January 2017.