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Samples in periodicals archive:
"Peripheral" tetrahydrobiopterin deficiency with hyperphenylalaninaemia due to incomplete 6-pyruvoyl tetrahydropterin synthase deficiency or heterozygosity. Niederwieser A, Shintaku H, Leimbacher W, Curtius HC, Hyànek J, Zeman J, Endres W.
Niederwieser A. et al. 'Peripheral' tetrahydrobiopterin deficiency with hyperphenylalaninaemia due to incomplete 6-pyruvoyl tetrahydropterin synthase.
OTHER VITAMINS / MINERALS - Ascorbates assist with folate and iron metabolism. 55,56 Furthermore, these nutrients contain mineral salts – magnesium and zinc – which are also known to play a role in the folate cycle. Defective 6-pyruvoyl tetrahydropterin synthase is known to impair tetrahydrobiopterin synthesis. The enzyme 6-pyruvoyl tetrahydropterin synthase consists of four identical subunits which convert dihydroneopterin triphosphate to 6-pyruvoyl-tetrahydropterin in the presence of magnesium.
660. Bürgisser, D. M. , Thöny, B. , Redweik, U. , Hess, D. , Heizmann, C. W. , Huber, R. and Nar, H. (1995). ”6-pyruvoyl tetrahydropterin synthase. An enzyme with a novel type of active site involving both zinc binding and an intersubunit catalytic triad motif; site-directed mutagenesis of the proposed active center, characterization of the metal binding site and modelling of substrate binding. ” J. Mol.