PEDIATRICS Vol. 70 No. 3 September 1982, pp. 376-380
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Use of Tetrahydropterins in the Treatment of Hyperphenylalaninemia due to Defective Synthesis of Tetrahydrobiopterin: Evidence that Peripherally Administered Tetrahydropterins Enter the Brain

Seymour Kaufman PhD1, Gregory Kapatos PhD1, Roderick R. Mclnnes MD, PhD1, Joseph D. Schulman MD1, and William B. Rizzo MD1

1 Laboratory of Neurochemistry, National Institute of Mental Health and Section on Human Biochemical Genetics, National Institute of Child Health and Human Development, Bethesda, Maryland; and Departments of Genetics and Pediatrics, The Hospital for Sick Children, Toronto

Substantial amounts of tetrahydrobiopterin and 6-methyltetrahydropterin can be detected in CSF when these pterins are given peripherally to patients with hyperphenylalaninemia due to defective biopterin sunthesis. Results of this study suggest that administration of either of these pterins in proper doses may prove to be a treatment not only for the impaired peripheral phenylalamne metabolism, but also for the neurologic disorders that are characteristic of the variant forms of hyperphenylalaninemia due to defective tetrahydrobiopterin synthesis or metabolism.

Submitted on September 29, 1981
Accepted on November 30, 1981




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