Gene:
molybdenum cofactor synthesis 2
Gene ID:
199464
Symbol:
MOCS2
Species:
1000
Description:
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. A bicistronic transcript encodes both the large and small subunits of molybdopterin synthase in two overlapping ORFs. The downstream ORF is thought to be translated by leaky scanning by ribosomes. A monocistronic transcript, in which only the downstream ORF is complete, also exists. Mutations in both ORFs have been associated with molybdenum cofactor deficiency B.