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Literature DB >> 6790841

Pterin metabolism in normal subjects and hyperphenylalaninaemic patients.

J L Dhondt, J P Farriaux, C Largilliere, M Dautrevaux, P Ardouin.

Abstract

The application of high performance liquid chromatography to the estimation of urinary pterins is illustrated by results from normal subjects and from patients with phenylketonuria, dihydropteridine reductase deficiency and biopterin synthetase deficiency. In normal subjects following a phenylalanine load the is a temporary increase in pterin elimination, the pattern being different to that seen in chronic hyperphenylalaninaemia.

Entities: Chemical Disease Species

Mesh：

Substances：

Year: 1981 PMID： 6790841 DOI： 10.1007/BF02263584

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

11 in total

1. Malignant hyperphenylalaninaemia--current status (June 1977).

Authors: D M Danks; K Bartholomé; B E Clayton; H Curtius; H Gröbe; S Kaufman; R Leeming; W Pfleiderer; H Rembold; F Rey
Journal: J Inherit Metab Dis Date: 1978 Impact factor: 4.982

2. [Hyperphenylalaninaemia with normal phenylalanine-hydroxylase activity and a deficiency of tetrahydrobiopterin and dihydropteridine reductase].

Authors: F Rey; J P Harpey; R J Leeming; J A Blair; J Aicardi; J Rey
Journal: Arch Fr Pediatr Date: 1977 Aug-Sep

3. Biopterin. V. De novo synthesis of dihydrobiopterin: evidence for its quinonoid structure and lack of dependence of its reduction to tetrahydrobiopterin on dihydrofolate reductase.

Authors: E M Gál; J A Bybee; A D Sherman
Journal: J Neurochem Date: 1979-01 Impact factor: 5.372

4. Analysis of reduced forms of biopterin in biological tissues and fluids.

Authors: T Fukushima; J C Nixon
Journal: Anal Biochem Date: 1980-02 Impact factor: 3.365

5. Biopterin derivatives in normal and phenylketonuric patients after oral loads of L-phenylalanine, L-tyrosine, and L-tryptophan.

Authors: R J Leeming; J A Blair; A Green; D N Raine
Journal: Arch Dis Child Date: 1976-10 Impact factor: 3.791

6. Separation of unconjugated pteridines by high-pressure cation-exchange liquid chromatography.

Authors: B Stea; R M Halpern; R A Smith
Journal: J Chromatogr Date: 1979-01-21

7. Hyperphenylalaninemia due to dihydropteridine reductase deficiency: diagnosis by measurement of oxidized and reduced pterins in urine.

Authors: S Milstien; S Kaufman; G K Summer
Journal: Pediatrics Date: 1980-04 Impact factor: 7.124

Pterin metabolism in normal subjects and hyperphenylalaninaemic patients.

1. Malignant hyperphenylalaninaemia--current status (June 1977).

2. [Hyperphenylalaninaemia with normal phenylalanine-hydroxylase activity and a deficiency of tetrahydrobiopterin and dihydropteridine reductase].

3. Biopterin. V. De novo synthesis of dihydrobiopterin: evidence for its quinonoid structure and lack of dependence of its reduction to tetrahydrobiopterin on dihydrofolate reductase.

4. Analysis of reduced forms of biopterin in biological tissues and fluids.

5. Biopterin derivatives in normal and phenylketonuric patients after oral loads of L-phenylalanine, L-tyrosine, and L-tryptophan.

6. Separation of unconjugated pteridines by high-pressure cation-exchange liquid chromatography.

7. Hyperphenylalaninemia due to dihydropteridine reductase deficiency: diagnosis by measurement of oxidized and reduced pterins in urine.

8. Atypical phenylketonuria caused by 7, 8-dihydrobiopterin synthetase deficiency.

9. Diagnosis of variants of hyperphenylalaninemia by determination of pterins in urine.

10. Hyperphenylalaninemia due to a deficiency of biopterin. A variant form of phenylketonuria.

1. Hepatic phenylalanine hydroxylase activity in hyperphenylalaninaemia.

2. Dihydropteridine reductase deficiency: non-response to oral tetrahydrobiopterin load test.