sepiapterin reductase

sepiapterin reductase
se·pi·ap·ter·in re·duc·tase (se″pe-apґtər-in re-dukґtās) [EC 1.1.1.153] an enzyme of the oxidoreductase class that catalyzes the final two-step, NADPH-dependent, reduction reaction in the biosynthesis of tetrahydrobiopterin (BH4), acting on the 6-pyruvoyltetrahydropterin intermediate formed by 6-pyruvoyltetrahydropterin synthase. It also catalyzes a step in the alternative salvage pathway of BH4 synthesis, and the reduction of various other carbonyl substances. Deficiency of the enzyme, an autosomal recessive trait, leads to BH4 and neurotransmitter deficiencies without hyperphenylalaninemia, manifesting as dopa-responsive dystonia.

Medical dictionary. 2011.

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