Details

Autor(en) / Beteiligte

• Werner, Ernst R.
• Werner-Felmayer, Gabriele
• Wachter, Helmut

Titel

High-performance liquid chromatographic methods for the quantification of tetrahydrobiopterin biosynthetic enzymes

Ist Teil von

• Journal of Chromatography B: Biomedical Sciences and Applications, 1996-09, Vol.684 (1), p.51-58

Ort / Verlag

Netherlands: Elsevier B.V

Erscheinungsjahr

1996

Link zum Volltext

Quelle

Elsevier Journal Backfiles on ScienceDirect (DFG Nationallizenzen)

Beschreibungen/Notizen

• Tetrahydrobiopterin is a cofactor in hydroxylation reactions, including phenylalanine 4-monooxygenase, tyrosine 3-monooxygenase, tryptophan 5-monooxygenase, allkyl glycol ether monooxygenase and nitric oxide synthase. Determination of its biosynthesis is carried out to diagnose inherited diseases leading to partial defects in tetrahydrobiopterin synthesis. In addition, tetrahydrobiopterin synthesis is induced by proinflammatory cytokines, and intracellular levels of tetrahydrobiopterin in many cases limit the activity of tetrahydropbiopterin-dependent reactions, such as nitric ozide synthase in intact cells. Biosynthesis of tetrahydrobiopterin from guanosine 5′-triphosphate (GTP) requires the action of three enzymes, GTP-cyclohydrolase I (E.C. 3.5.4.16), 6-pyruvoyl tetrahydropterin synthase (E.C. 4.6.1.10) and sepiapterin reductase (E.C. 1.1.1.153). Methods for quantification of biopterin and related pteridines in biological matrices by HPLC and application of these for determining the activity of the three tetrahydrobiopterin biosynthetic enzymes are reviewed in this article.