Molybdopterin synthase

Molybdopterin synthase
Identifiers
EC no.2.8.1.12
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IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
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Molybdopterin synthase (EC 2.8.1.12, MPT synthase) is an enzyme required to synthesize molybdopterin (MPT) from precursor Z (now known as cyclic pyranopterin monophosphate). Molydopterin is subsequently complexed with molybdenum to form molybdenum cofactor (MoCo). MPT synthase catalyses the following chemical reaction:

precursor Z + 2 [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH2-C(O)SH + H2O molybdopterin + 2 molybdopterin-synthase sulfur-carrier protein

Molybdopterin synthase is heterodimeric and coded for by the MOCS2 gene. Genetic deficiencies of enzymes such as MPT synthase, which are involved in MoCo biosynthesis, lead to MoCo deficiency, a rare disease that results in severe neurological abnormalities.