Literature DB >> 2079837

Relationship between plasma and red cell biopterins in acute and chronic hyperphenylalaninaemia.

R J Leeming1, S K Hall, I M Surplice, A Green.   

Abstract

Biopterin concentrations in red blood cells were higher in patients with chronic hyperphenylalaninaemia than following a single oral dose of phenylalanine, although the plasma concentrations were similar. These findings suggest that tetrahydrobiopterin is synthesized in immature red blood cells in response to hyperphenylalaninaemia. The less marked increase in plasma biopterin suggests slow release from a tissue reservoir. The maintenance of a differential between erythrocyte and plasma biopterin concentrations suggests that some mechanism retains biopterin intracellularly.

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Year:  1990        PMID： 2079837     DOI： 10.1007/BF01800214

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 derivatives in human body fluids and tissues.

Authors:  R J Leeming; J A Blair; V Melikian; D J O'Gorman
Journal:  J Clin Pathol       Date:  1976-05       Impact factor: 3.411

4.  Proceedings: Intestinal absorption of tetrahydrobiopterin and pterolic acid in man: a comparison of models for the transport of folates across the small intestine.

Authors:  J A Blair; K Ratanasthien; R J Leeming
Journal:  Gut       Date:  1974-10       Impact factor: 23.059

5.  Blood spots on Guthrie cards can be used for inherited tetrahydrobiopterin deficiency screening in hyperphenylalaninaemic infants.

Authors:  R J Leeming; P A Barford; J A Blair; I Smith
Journal:  Arch Dis Child       Date:  1984-01       Impact factor: 3.791

6.  Developmental aspects of pteridine metabolism and relationships with phenylalanine metabolism.

Authors:  J L Dhondt; P Ardouin; J M Hayte; J P Farriaux
Journal:  Clin Chim Acta       Date:  1981-10-26       Impact factor: 3.786

7.  The effects of pathological and normal physiological processes on biopterin derivative levels in man.

Authors:  R J Leeming; J A Blair
Journal:  Clin Chim Acta       Date:  1980-11-20       Impact factor: 3.786

8.  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

9.  HPLC measurement of phenylalanine in plasma.

Authors:  N D Atherton; A Green
Journal:  Clin Chem       Date:  1988-11       Impact factor: 8.327

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

Authors:  S Kaufman; S Berlow; G K Summer; S Milstien; J D Schulman; S Orloff; S Spielberg; S Pueschel
Journal:  N Engl J Med       Date:  1978-09-28       Impact factor: 91.245
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  3 in total

1.  International database of tetrahydrobiopterin deficiencies.

Authors:  N Blau; I Barnes; J L Dhondt
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982

2.  Altered tetrahydrobiopterin metabolism in patients with phenylalanine hydroxylase deficiency.

Authors:  Francesca Nardecchia; Flavia Chiarotti; Claudia Carducci; Silvia Santagata; Giulia Valentini; Antonio Angeloni; Nenad Blau; Vincenzo Leuzzi
Journal:  Eur J Pediatr       Date:  2017-05-24       Impact factor: 3.183

3.  Analysis of plasma biopterin levels in psychiatric disorders suggests a common BH4 deficit in schizophrenia and schizoaffective disorder.

Authors:  Mary Ann Richardson; Laura L Read; Margaret A Reilly; James D Clelland; Catherine L Taylor Clelland
Journal:  Neurochem Res       Date:  2006-12-09       Impact factor: 3.996
  3 in total

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