molybdopterin synthase catalytic subunit; molybdenum cofactor synthesis protein 2B; MOCS2B; molybdenum cofactor synthesis protein 2 large subunit; molybdopterin-synthase large subunit; MPT synthase large subunit; MOCO1-B (MOCS2, MCBPE, MOCO1)

Function

• 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
• cofactor biosynthesis; molybdopterin biosynthesis

Structure

• belongs to the moaE family, MOCS2B subfamily

Compartment

• cytoplasm, cytosol

Expression

• highest levels are found in heart & skeletal muscle
• lower levels are present in brain, kidney & pancreas
• very low levels are found in lung & peripheral blood leukocytes

Pathology

• defects in MOCS2 are the cause of molybdenum cofactor deficiency type B

Genetics

• MOCS2 is produced by a bicistronic gene which also produces the small subunit (MOCS2A) from an overlapping reading frame
• expression of these 2 proteins are related since a mutation that removes the start codon of the small subunit (MOCS2A) also impairs expression of the large subunit (MOCS2B)

More general terms

• molybdenum cofactor synthesis protein
• enzyme catalytic subunit
• phosphoprotein

Additional terms

• molybdenum cofactor deficiency

Component of

• molybdopterin synthase complex

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=4338
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4338
• OMIM: https://mirror.omim.org/entry/252150
• OMIM: https://mirror.omim.org/entry/603708
• UniProt: http://www.uniprot.org/uniprot/O96007.html