4ap8
From Proteopedia
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{{STRUCTURE_4ap8| PDB=4ap8 | SCENE= }} | {{STRUCTURE_4ap8| PDB=4ap8 | SCENE= }} | ||
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===Crystal structure of human Molybdopterin synthase catalytic subunit (MOCS2B)=== | ===Crystal structure of human Molybdopterin synthase catalytic subunit (MOCS2B)=== | ||
| + | ==Disease== | ||
| + | [[http://www.uniprot.org/uniprot/MOC2B_HUMAN MOC2B_HUMAN]] Molybdenum cofactor deficiency type B (MOCOD type B) [MIM:[http://omim.org/entry/252150 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. | ||
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| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/MOC2B_HUMAN 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.<ref>PMID:12732628</ref> <ref>PMID:15073332</ref> | ||
==About this Structure== | ==About this Structure== | ||
[[4ap8]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4AP8 OCA]. | [[4ap8]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4AP8 OCA]. | ||
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| + | ==Reference== | ||
| + | <references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Molybdopterin synthase]] | [[Category: Molybdopterin synthase]] | ||
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[[Category: Bountra, C.]] | [[Category: Bountra, C.]] | ||
[[Category: Burgess-Brown, N.]] | [[Category: Burgess-Brown, N.]] | ||
| - | [[Category: Delft, F | + | [[Category: Delft, F von.]] |
[[Category: Edwards, A.]] | [[Category: Edwards, A.]] | ||
[[Category: Froese, D S.]] | [[Category: Froese, D S.]] | ||
Revision as of 18:22, 19 June 2013
Contents |
Crystal structure of human Molybdopterin synthase catalytic subunit (MOCS2B)
Disease
[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
[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.[1] [2]
About this Structure
4ap8 is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- ↑ 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
