7-Substituted pterins: Formation during phenylalanine hydroxylation in the absence of dehydratase

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1990
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Curtius, Hans-Christoph
Adler, Carmen
Rebrin, Igor
Heizmann, Claus
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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 Biowissenschaften, Biologie
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ISO 690CURTIUS, 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. 1990, 172(3), pp. 1060-1066. ISSN 0006-291X. Available under: doi: 10.1016/0006-291X(90)91554-6
BibTex
@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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