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6-Pyruvoyl tetrahydrobiopterin synthase (PTPS) is an enzyme that catalyzes the second step in the de novo biosynthesis of tetrahydrobiopterin (BH4), a critical cofactor for several essential metabolic processes. PTPS converts 7,8-dihydroneopterin triphosphate into 6-pyruvoyltetrahydropterin with the elimination of inorganic triphosphate. Mutations in PTPS can lead to hyperphenylalaninemia due to BH4 deficiency.
Catalyzes the conversion of 7,8-dihydroneopterin triphosphate to 6-pyruvoyltetrahydropterin
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