| Primary Identifier | IPR028888 | Type | Family |
| Short Name | MOCS2B_euk |
| 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 []. |
