Literature DB >> 3939528

Liver enzyme activities in hyperphenylalaninaemia due to a defective synthesis of tetrahydrobiopterin.

J L Dhondt, R G Cotton, D M Danks.   

Abstract

This report confirms the accumulation of neopterin and the low biopterin concentration in the liver of a "biopterin-synthetase" deficient patient. Enzymatic studies suggest that the defect lies between the "X"-compound (6-pyruvoyl-tetrahydropterin?) and tetrahydrobiopterin and that pterin found in the patient's liver arises from the "X"-compound.

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Year:  1985        PMID： 3939528     DOI： 10.1007/bf01801660

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


  14 in total

1.  Biosynthesis of tetrahydrobiopterin: possible involvement of tetrahydropterin intermediates.

Authors:  D Heintel; S Ghisla; H C Curtius; A Niederwieser; R A Levine
Journal:  Neurochem Int       Date:  1984       Impact factor: 3.921

2.  Guanosine triphosphate cyclohydrolase activity in rat tissues.

Authors:  Z Bellahsene; J L Dhondt; J P Farriaux
Journal:  Biochem J       Date:  1984-01-01       Impact factor: 3.857

3.  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

4.  Intermediates in the enzymic synthesis of tetrahydrobiopterin in Drosophila melanogaster.

Authors:  A C Switchenko; J P Primus; G M Brown
Journal:  Biochem Biophys Res Commun       Date:  1984-05-16       Impact factor: 3.575

Review 5.  Tetrahydrobiopterin deficiencies: preliminary analysis from an international survey.

Authors:  J L Dhondt
Journal:  J Pediatr       Date:  1984-04       Impact factor: 4.406

6.  Two new tetrahydropterin intermediates in the adrenal medullary de novo biosynthesis of tetrahydrobiopterin.

Authors:  G K Smith; C A Nichol
Journal:  Biochem Biophys Res Commun       Date:  1984-05-16       Impact factor: 3.575

7.  Guanosine triphosphate cyclohydrolase I assay in human and rat liver using high-performance liquid chromatography of neopterin phosphates and guanine nucleotides.

Authors:  N Blau; A Niederwieser
Journal:  Anal Biochem       Date:  1983-02-01       Impact factor: 3.365

8.  Purification and characterization of sepiapterin reductase from rat erythrocytes.

Authors:  T Sueoka; S Katoh
Journal:  Biochim Biophys Acta       Date:  1982-08-06

9.  Malignant hyperphenylalaninemia--clinical features, biochemical findings, and experience with administration of biopterins.

Authors:  D M Danks; P Schlesinger; F Firgaira; R G Cotton; B M Watson; H Rembold; G Hennings
Journal:  Pediatr Res       Date:  1979-10       Impact factor: 3.756

10.  Dihydropteridine-reductase activity in breast cancer. Preliminary report.

Authors:  J L Dhondt; J Bonneterre; J P Farriaux; J Lefebvre; A Demaille
Journal:  Biomedicine       Date:  1981-05
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