Literature DB >> 3088325

A model for hyperphenylalaninaemia due to tetrahydrobiopterin deficiency.

R G Cotton.   

Abstract

A model for tetrahydrobiopterin deficiency in mice is described. Elevated levels of phenylalanine produced in the model were shown to be dramatically reduced after injection of tetrahydrobiopterin. A comparison of several reduced pterins for their efficacy in the system is described. The unnatural S isomer of tetrahydrobiopterin was shown to be active in the system.

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Year:  1986        PMID： 3088325     DOI： 10.1007/bf01813895

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


  19 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.  Atypical phenylketonuria due to tetrahydrobiopterin deficiency. Diagnosis and treatment with tetrahydrobiopterin, dihydrobiopterin and sepiapterin.

Authors:  H C Curtius; A Niederwieser; M Viscontini; A Otten; J Schaub; S Scheibenreiter; H Schmidt
Journal:  Clin Chim Acta       Date:  1979-04-16       Impact factor: 3.786

3.  Treatment of dystonia with tetrahydrobiopterin.

Authors:  P A LeWitt; R P Newman; L P Miller; W Lovenberg; R Eldridge
Journal:  N Engl J Med       Date:  1983-01-20       Impact factor: 91.245

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.  Letter: Tetrahydrobiopterin treatment of variant form of phenylketonuria.

Authors:  D M Danks; R G Cotton; P Schlesinger
Journal:  Lancet       Date:  1975-11-22       Impact factor: 79.321

6.  Biopterin. VII. Inhibition of synthesis of reduced biopterins and its bearing on the function of cerebral tryptophan-5-hydroxylase in vivo.

Authors:  E M Gál; D H Whitacre
Journal:  Neurochem Res       Date:  1981-03       Impact factor: 3.996

7.  6,6-Dimethylpterins: stable quinoid dihydropterin substrate for dihydropteridine reductase and tetrahydropterin cofactor for phenylalanine hydroxylase.

Authors:  S W Bailey; J E Ayling
Journal:  Biochemistry       Date:  1983-04-12       Impact factor: 3.162

8.  Hydroxylase cofactor activity in cerebrospinal fluid of normal subjects and patients with Parkinson's disease.

Authors:  W Lovenberg; R A Levine; D S Robinson; M Ebert; A C Williams; D B Calne
Journal:  Science       Date:  1979-05-11       Impact factor: 47.728

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

Authors:  A Niederwieser; H C Curtius; O Bettoni; J Bieri; B Schircks; M Viscontini; J Schaub
Journal:  Lancet       Date:  1979-01-20       Impact factor: 79.321

10.  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
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  1 in total

1.  A requirement for Gch1 and tetrahydrobiopterin in embryonic development.

Authors:  Gillian Douglas; Ashley B Hale; Mark J Crabtree; Brent J Ryan; Alex Hansler; Katrin Watschinger; Steven S Gross; Craig A Lygate; Nicholas J Alp; Keith M Channon
Journal:  Dev Biol       Date:  2014-12-31       Impact factor: 3.582
  1 in total

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