Tetrahydrobiopterin
Template:Chembox new Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: 5,6,7,8-tetrahydrobiopterin, BH4
Overview
Tetrahydrobiopterin is a cofactor in the synthesis of nitric oxide. It is also essential in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine to L-dopa by the enzyme tyrosine hydroxylase; and conversion of tryptophan to 5-hydroxytryptophan via tryptophan hydroxylase.
Clinical significance
A defect in BH4 production and/or a defect in the enzyme dihydropteridine reductase (DHPR) causes phenylketonuria type IV, as well as dopa-responsive dystonias.
Synthesis
Tetrahydrobiopterin is synthesized from GTP via two intermediates.
References
Thony B, Auerbach G, Blau N (2000). "Tetrahydrobiopterin biosynthesis, regeneration and functions". Biochem J. 347 Pt 1: 1–16. PMID 10727395.
See also
- Tetrahydrobiopterin deficiency This can also cause mental retardation as seen in patients with Phenylketonuria (PKU).
External links
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