Cyclic pyranopterin monophosphate

In 2010, a baby girl was diagnosed with molybdenum cofactor deficiency at 6 days old. After treatment with purified cyclic pyranopterin monophosphate (cPMP), the precursor of MoCo whose synthesis was defective, starting at 36 days old, all urinary metabolites of sulfite oxidase and xanthine oxidase deficiency returned to almost normal values and has remained constant for more than one year. 

The biosynthesis of Moco starts with GTP, which is converted in a GTP cyclohydrolase-independent reaction to the first stable intermediate precursor Z, a cyclic pyranopterin monophosphate. Precursor Z is converted into MPT by the insertion of two sulfur atoms, forming the dithiolene group which coordinates the molybdenum atom. After insertion of molybdate, Moco is formed that can be further modified and is inserted into various molybdoenzymes. ... 

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