@article{Adler19927Subs-7681,
  year={1992},
  doi={10.1111/j.1432-1033.1992.tb17167.x},
  title={7-Substituted pterins in humans with suspected pterin-4a-carbinolamine dehydratase deficiency : mechanism of formation via non-enzymatic transformation from 6-substituted pterins},
  number={1},
  volume={208},
  issn={0014-2956},
  journal={European Journal of Biochemistry},
  pages={139--144},
  author={Adler, Carmen and Ghisla, Sandro and Rebrin, Igor and Haavik, Jan and Heizmann, Claus W. and Blau, Nenad and Kuster, Thomas and Curtius, Hans-Christoph}
}

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    <dcterms:issued>1992</dcterms:issued>
    <dc:contributor>Kuster, Thomas</dc:contributor>
    <dc:contributor>Rebrin, Igor</dc:contributor>
    <dcterms:bibliographicCitation>First publ. in: European Journal of Biochemistry 208 (1992), 1, pp. 139-144</dcterms:bibliographicCitation>
    <dc:contributor>Ghisla, Sandro</dc:contributor>
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    <dc:contributor>Curtius, Hans-Christoph</dc:contributor>
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    <dc:contributor>Haavik, Jan</dc:contributor>
    <dcterms:abstract xml:lang="eng">A recently described new form of hyperphenylalaninemia is characterized by the excretion of 7-substituted isomers of biopterin and neopterin and 7-oxo-biopterin in the urine of patients. It has been shown that the 7-substituted isomers of biopterin and neopterin derive from L-tetrahydrobiopterin and D-tetrahydroneopterin and are formed during hydroxylation of phenylalanine to tyrosine with rat liver dehydratase-free phenylalanine hydroxylase.&lt;br /&gt;We have now obtained identical results using human phenylalanine hydroxylase. The identity of the pterin formed in vitro and derived from L-tetrahydrobiopterin as 7-(1',2'-dihydroxypropyl)pterin was proven by gas-chromatography mass spectrometry. Tetrahydroneopterin and 6-hydroxymethyltetrahydropterin also are converted to their corresponding 7-substituted isomers and serve as cofactors in the phenylalanine hydroxylase reaction. Dihydroneopterin is converted by dihydrofolate reductase to the tetrahydro form which is biologically active as a cofactor for the aromatic amino acid monooxygenases. The 6-substituted pterin to 7-substituted pterin conversion occurs in the absence of pterin-4a-carbinolamine dehydratase and is shown to be a nonenzymatic process. 7-Tetrahydrobiopterin is both a substrate (cofactor) and a competitive inhibitor with 6-tetrahydrobiopterin (Ki ≈ 8 μM) in the phenylalanine hydroxylase reaction. For the first time, the formation of 7-substituted pterins from their 6-substituted isomers has been demonstrated with tyrosine hydroxylase, another important mammalian enzyme which functions in the hydroxylation of phenylalanine and tyrosine.</dcterms:abstract>
    <dc:creator>Rebrin, Igor</dc:creator>
    <dc:creator>Curtius, Hans-Christoph</dc:creator>
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    <dc:contributor>Blau, Nenad</dc:contributor>
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    <dc:creator>Adler, Carmen</dc:creator>
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