Tetrahydrobiopterin as an Alternative Treatment for Mild Phenylketonuria
- 26 December 2002
- journal article
- research article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 347 (26) , 2122-2132
- https://doi.org/10.1056/nejmoa021654
Abstract
Hyperphenylalaninemia is a common inherited metabolic disease that is due to phenylalanine hydroxylase deficiency, and at least half the affected patients have mild clinical phenotypes. Treatment with a low-phenylalanine diet represents a substantial psychosocial burden, but alternative treatments have not been effective.Keywords
This publication has 40 references indexed in Scilit:
- Tetrahydrobiopterin biosynthesis, regeneration and functionsBiochemical Journal, 2000
- A European Multicenter Study of Phenylalanine Hydroxylase Deficiency: Classification of 105 Mutations and a General System for Genotype-Based Prediction of Metabolic PhenotypeAmerican Journal of Human Genetics, 1998
- An ongoing debate over phenylalanine hydroxylase deficiency in phenylketonuria.Journal of Clinical Investigation, 1998
- Modulation of Inducible Nitric Oxide Synthase mRNA Stability by Tetrahydrobiopterin in Vascular Smooth Muscle CellsBiochemical and Biophysical Research Communications, 1998
- Human Phenylalanine Hydroxylase Mutations and Hyperphenylalaninemia Phenotypes: A Metanalysis of Genotype-Phenotype CorrelationsAmerican Journal of Human Genetics, 1997
- SWISS‐MODEL and the Swiss‐Pdb Viewer: An environment for comparative protein modelingElectrophoresis, 1997
- The Phenylalanine Hydroxylating SystemPublished by Wiley ,1993
- Fetal damage due to maternal phenylketonuria: Effects of dietary treatment and maternal phenylalanine concentrations around the time of conceptionJournal of Inherited Metabolic Disease, 1990
- Clinical role of pteridine therapy in tetrahydrobiopterin deficiencyJournal of Inherited Metabolic Disease, 1985
- Geometric method for measuring body surface area: A height-weight formula validated in infants, children, and adultsThe Journal of Pediatrics, 1978