7-Substituted pterins: Formation during phenylalanine hydroxylation in the absence of dehydratase
7-Substituted pterins: Formation during phenylalanine hydroxylation in the absence of dehydratase
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1990
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Biochemical and biophysical research communications ; 172 (1990), 3. - pp. 1060-1066. - ISSN 0006-291X
Abstract
Previously we described a new form of human hyperphenylalaninemia characterized by the formation of 7-substituted pterins. We present evidence strongly suggesting that the 7-substituted pterins are formed by rearrangement of 6-substituted pterins. This rearrangement occurs during the phenylalanine hydroxylase reaction cycle which normally involves the enzymes phenylalanine hydroxylase, pterin-4a-OH-dehydratase, and q-dihydropterin reductase, specifically in the absence of dehydratase activity. We conclude that formation of 7-substituted pterins in humans is a consequence of an absence of dehydratase activity, which might result from a genetic defect. A chemical mechanism for this rearrangement is presented. Our results also suggest that tetrahydroneopterin can be a cofactor for the phenylalanine hydroxylase system in vivo.
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570 Biosciences, Biology
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CURTIUS, Hans-Christoph, Carmen ADLER, Igor REBRIN, Claus HEIZMANN, Sandro GHISLA, 1990. 7-Substituted pterins: Formation during phenylalanine hydroxylation in the absence of dehydratase. In: Biochemical and biophysical research communications. 172(3), pp. 1060-1066. ISSN 0006-291X. Available under: doi: 10.1016/0006-291X(90)91554-6BibTex
@article{Curtius19907Subs-6890, year={1990}, doi={10.1016/0006-291X(90)91554-6}, title={7-Substituted pterins: Formation during phenylalanine hydroxylation in the absence of dehydratase}, number={3}, volume={172}, issn={0006-291X}, journal={Biochemical and biophysical research communications}, pages={1060--1066}, author={Curtius, Hans-Christoph and Adler, Carmen and Rebrin, Igor and Heizmann, Claus and Ghisla, Sandro} }
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