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

Journal of Chromatography. B, Biomedical Applications
E R WernerH Wachter


Tetrahydrobiopterin is a cofactor in hydroxylation reactions, including phenylalanine 4-monooxygenase, tyrosine 3-monooxygenase, tryptophan 5-monooxygenase, alkyl 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 tetrahydro-biopterin in many cases limit the activity of tetrahydrobiopterin-dependent reactions, such as nitric oxide synthase in intact cells. Biosynthesis of tetrahydrobiopterin from guanosine 5'-triphosphate (GTP) requires the action of three enzymes, GTP-cyclohydrolase I (E.C., 6-pyruvoyl tetrahydropterin synthase (EC, and sepiapterin reductase (E.C. 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.


Dec 1, 1992·European Journal of Biochemistry·G SchoedonN Blau
Dec 1, 1990·The Journal of Experimental Medicine·G Werner-FelmayerH Wachter
Aug 1, 1990·Proceedings of the National Academy of Sciences of the United States of America·B A CitronS Kaufman
Jan 1, 1989·Biomedicine & Pharmacotherapy = Biomédecine & Pharmacothérapie·G ReibneggerH Wachter
Aug 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·K TanakaS Milstien
Jan 1, 1985·Annual Review of Biochemistry·C A NicholD S Duch
Jan 1, 1988·Annual Review of Nutrition·N Blau
May 15, 1985·Clinica Chimica Acta; International Journal of Clinical Chemistry·N BlauA Niederwieser
Jun 15, 1988·Clinica Chimica Acta; International Journal of Clinical Chemistry·J Ferre, E W Naylor
Nov 13, 1987·Biochemical and Biophysical Research Communications·J Ferré, E W Naylor
Jan 1, 1987·Biomedical Chromatography : BMC·J D DewitteH H Floch
Jun 16, 1988·Biochemical and Biophysical Research Communications·H C CurtiusJ L Dhondt
Jan 1, 1985·Journal of Cellular Biochemistry·I Ziegler
Apr 20, 1983·Biochimica Et Biophysica Acta·S I YoshiokaM Akino
Feb 1, 1980·Analytical Biochemistry·T Fukushima, J C Nixon
Aug 4, 1980·Clinica Chimica Acta; International Journal of Clinical Chemistry·H RokosH J Kirstaedter
Jan 17, 1995·Biochemical and Biophysical Research Communications·J M WoodM L Gardner
Jan 1, 1993·Annual Review of Nutrition·S Kaufman
May 1, 1993·Proceedings of the Society for Experimental Biology and Medicine·E R WernerH Wachter
Apr 21, 1993·Journal of Chromatography·M CanditoP Chambon
Jan 1, 1996·Reviews of Physiology, Biochemistry and Pharmacology·E R WernerB Mayer

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Jan 20, 2004·Biochimica Et Biophysica Acta·Lina ElzaoukBeat Thöny
Aug 28, 2001·Brain Research. Brain Research Protocols·Y S KanekoA Ota
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