Protein Domain : IPR028888

Type:  Family Name:  Molybdopterin synthase catalytic subunit, eukaryotes
Description:  Eukaryotic molybdopterin synthase, like the Escherichia coli MoaD-MoaE counterpart, is a heterotetramer that is composed of two subunits: MOCS2A and MOCS2B. MOCS2B is the catalytic subunit of the molybdopterin synthase complex (MOCS2), a complex that catalyses the conversion of precursor Z into molybdopterin. MOC2SB acts by mediating the incorporation of two sulfur atoms from thiocarboxylated MOCS2A (the small MOCS2 subunit which functions as a sulfur carrier) into precursor Z to generate a dithiolene group [, , , ].Mutations in human MOCS2B gene cause Molybdenum cofactor deficiency type B (MOCOD type B), an autosomal recessive disease which leads to the pleiotropic loss of all molybdoenzyme activities and is characterised by severe neurological damage, neonatal seizures and early childhood death []. Short Name:  MOCS2B_euk
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0 Child Features

0 Contains

1 Cross References

Identifier
MF_03052

0 Found In

4 GO Annotations

GO Term Gene Name
GO:0030366 IPR028888
GO:0006777 IPR028888
GO:0005829 IPR028888
GO:0019008 IPR028888

4 Ontology Annotations

GO Term Gene Name
GO:0030366 IPR028888
GO:0006777 IPR028888
GO:0005829 IPR028888
GO:0019008 IPR028888

1 Parent Features

DB identifier Type Name
IPR003448 Family Molybdopterin biosynthesis MoaE

0 Proteins

5 Publications

First Author Title Year Journal Volume Pages PubMed ID
            15073332
            10053004
            16737835
            12732628
            10383438