Eukaryotic molybdoenzymes use a unique molybdenum cofactor (MoCo) consisting of a pterin, termed molybdopterin, and the catalytically active metal molybdenum. MoCo is synthesized from precursor Z by the heterodimeric enzyme molybdopterin synthase. The large and small subunits of molybdopterin synthase are both encoded from this gene by overlapping open reading frames. The proteins were initially thought to be encoded from a bicistronic transcript. They are now thought to be encoded from monocistronic transcripts. Alternatively spliced transcripts have been found for this locus that encode the large and small subunits. [provided by RefSeq, Jul 2008]
Function:
The large (MOCS2B) and small (MOCS2A) subunits of molybdopterin synthase are both encoded from the MOCS2 gene by overlapping open reading frames. MOCS2B is the catalytic subunit of the molybdopterin synthase complex, a complex that catalyzes the conversion of precursor Z into molybdopterin. It acts by mediating the incorporation of 2 sulfur atoms from thiocarboxylated MOCS2A into precursor Z to generate a dithiolene group.
Subunit:
Heterotetramer; composed of 2 small (MOCS2A) and 2 large (MOCS2B) subunits.
Subcellular Location:
Cytoplasmic
Tissue Specificity:
Highest levels are found in heart and skeletal muscle. Lower levels are present in brain, kidney and pancreas.Very low levels are found in lung and peripheral blood leukocytes.
DISEASE:
Molybdenum cofactor deficiency, complementation group B(MOCODB)
Similarity:
Belongs to the MoaE family. MOCS2B subfamily.
SWISS:
O96007
Gene ID:
4338
Database links:
Entrez Gene: 4338 Human
Omim: 603708 Human
SwissProt: O96007 Human
Unigene: 163645 Human
|
|
