tetrahydrobiopterin

tetrahydrobiopterin
tet·ra·hy·dro·bi·op·ter·in (BH4) (BH4) (tet″rə-hi″dro-bi-opґtər-in) a reduced form of dihydrobiopterin that functions as a coenzyme in the reactions hydroxylating phenylalanine, tryptophan, and tyrosine by carrying electrons to oxygen. Defects in the biosynthesis or regeneration of the coenzyme affect all three hydroxylation reactions, interfere with production of the corresponding neurotransmitter precursors, and result in hyperphenylalaninemia unresponsive to a low-phenylalanine diet unless the missing neurotransmitters can also be supplied; see also tetrahydrobiopterin deficiency, under deficiency.

Medical dictionary. 2011.

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  • Tetrahydrobiopterin — Systematic (IUPAC) name (6R) 2 Amino 6 [(1R,2S) 1,2 dihydroxypropyl] 5,6,7,8 tetrahydropteridin 4(1H) one Clinical data …   Wikipedia

  • Tetrahydrobiopterin — Strukturformel Allgemeines Name Biopterin Andere Namen 2 Amino 6 (L erythro …   Deutsch Wikipedia

  • Tetrahydrobiopterin deficiency — Infobox Disease Name = PAGENAME Caption = Tetrahydrobiopterin DiseasesDB = ICD10 = ICD10|E|70|1|e|70 ICD9 = ICD9|270.1 OMIM = MedlinePlus = eMedicineSubj = ped eMedicineTopic = 2226 MeshID = Tetrahydrobiopterin deficiency (also known as BH4… …   Wikipedia

  • tetrahydrobiopterin deficiency — any of several disorders in the synthesis or regeneration of the cofactor tetrahydrobiopterin; clinical presentation is usually similar to phenylketonuria but because the cofactor also functions in the synthesis of various monoamine… …   Medical dictionary

  • tetrahydrobiopterin — noun A naturally occurring nutrient and essential cofactor of the three aromatic amino acid hydroxylase enzymes, used in the biosynthesis of the neurotransmitters serotonin, melatonin, dopamine, norepinephrine …   Wiktionary

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  • Phenylketonuria — PKU redirects here. For other uses, see PKU (disambiguation). Phenylketonuria Classification and external resources ICD 10 E70.0 ICD 9 …   Wikipedia

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