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.
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| 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][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
- ↑ Leimkuhler S, Freuer A, Araujo JA, Rajagopalan KV, Mendel RR. Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency. J Biol Chem. 2003 Jul 11;278(28):26127-34. Epub 2003 May 5. PMID:12732628 doi:10.1074/jbc.M303092200
- ↑ Leimkuhler S, Freuer A, Araujo JA, Rajagopalan KV, Mendel RR. Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency. J Biol Chem. 2003 Jul 11;278(28):26127-34. Epub 2003 May 5. PMID:12732628 doi:10.1074/jbc.M303092200
- ↑ Matthies A, Rajagopalan KV, Mendel RR, Leimkuhler S. Evidence for the physiological role of a rhodanese-like protein for the biosynthesis of the molybdenum cofactor in humans. Proc Natl Acad Sci U S A. 2004 Apr 20;101(16):5946-51. Epub 2004 Apr 8. PMID:15073332 doi:10.1073/pnas.0308191101
