Molybdopterin synthase catalytic subunit, eukaryotes (IPR028888)

Short name: MOCS2B_euk

Overlapping homologous superfamilies

Family relationships


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 [PMID: 12732628, PMID: 15073332, PMID: 16737835, PMID: 10383438].

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 [PMID: 10053004].

GO terms

Biological Process

GO:0006777 Mo-molybdopterin cofactor biosynthetic process

Molecular Function

GO:0030366 molybdopterin synthase activity

Cellular Component

GO:0005829 cytosol
GO:0019008 molybdopterin synthase complex

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.