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

Curtius, Hans-Christoph; Adler, Carmen; Rebrin, Igor; Heizmann, Claus; Ghisla, Sandro

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

Files

Biochem_Biophys_Res_Commun_1990_Curtius7_Substituted_pterins_for.pdf(415.28 KB)

Date

1990

Authors

Curtius, Hans-Christoph

Adler, Carmen

Rebrin, Igor

Heizmann, Claus

Ghisla, Sandro

Publication type

Journal article

Published in

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.

Subject (DDC)

570 Biosciences, Biology

Cite This

ISO 690

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)91554-6

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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