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Tetrahydrobiopterin
From Wikipedia, the free encyclopedia
| Tetrahydrobiopterin | |
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| Identifiers | |
| CAS number | [] |
| PubChem | |
| MeSH | |
| Properties | |
| Molecular formula | C9H15N5O3 |
| Molar mass | 241.247 |
| Except where noted otherwise, data are given for materials in their standard state (at 25 °C, 100 kPa) Infobox disclaimer and references |
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Tetrahydrobiopterin or BH4 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.
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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.
- Tetrahydrobiopterin deficiency This can also cause mental retardation as seen in patients with Phenylketonuria (PKU).
- Thony B, Auerbach G, Blau N (2000). "Tetrahydrobiopterin biosynthesis, regeneration and functions". Biochem J 347 Pt 1: 1-16. PMID 10727395. Full text
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