Literature DB >> 3382399

Primapterin, anapterin, and 6-oxo-primapterin, three new 7-substituted pterins identified in a patient with hyperphenylalaninemia.

H C Curtius1, T Kuster, A Matasovic, N Blau, J L Dhondt.   

Abstract

Three unknown compounds present in the urine of a patient with mild hyperphenylalaninemia were identified to be L-erythro-7-iso-biopterin, D-erythro-7-iso-neopterin, and L-erythro-6-oxo-7-iso-biopterin. The newly identified pterins were named primapterin, anapterin, and 6-oxo-primapterin, respectively. Primapterin and anapterin are present in very low concentrations in every human urine, as well as in the liver of man and mouse, whereas 6-oxo-primapterin was detected in the patient's urine only. Substantial amounts of primapterin were excreted in the patient described. The metabolic origin of primapterin and anapterin is still obscure.

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Year:  1988        PMID： 3382399     DOI： 10.1016/s0006-291x(88)81153-7

Source DB:  PubMed          Journal:  Biochem Biophys Res Commun        ISSN： 0006-291X            Impact factor:   3.575


  12 in total

1.  Lessons from 30 years of selective screening for tetrahydrobiopterin deficiency.

Authors:  Jean-Louis Dhondt
Journal:  J Inherit Metab Dis       Date:  2010-05-11       Impact factor: 4.982

2.  Hyperphenylalaninaemia presumably due to carbinolamine dehydratase deficiency: loading tests with pterin derivatives.

Authors:  N Blau; L Kierat; H C Curtius; M Blaskovics; T Giudici
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

3.  Suspected pterin-4a-carbinolamine dehydratase deficiency: hyperphenylalaninaemia due to inhibition of phenylalanine hydroxylase by tetrahydro-7-biopterin.

Authors:  C Adler; S Ghisla; I Rebrin; C W Heizmann; N Blau; H C Curtius
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

4.  Identity of 4a-carbinolamine dehydratase, a component of the phenylalanine hydroxylation system, and DCoH, a transregulator of homeodomain proteins.

Authors:  B A Citron; M D Davis; S Milstien; J Gutierrez; D B Mendel; G R Crabtree; S Kaufman
Journal:  Proc Natl Acad Sci U S A       Date:  1992-12-15       Impact factor: 11.205

5.  Prenatal diagnosis of atypical phenylketonuria.

Authors:  N Blau; A Niederwieser; H C Curtius; L Kierat; W Leimbacher; A Matasovic; F Binkert; H Lehmann; D Leupold; O Guardamagna
Journal:  J Inherit Metab Dis       Date:  1989       Impact factor: 4.982

6.  Primapterinuria: a new variant of atypical phenylketonuria.

Authors:  N Blau; H C Curtius; T Kuster; A Matasovic; G Schoedon; J L Dhondt; P Guibaud; T Giudici; M Blaskovics
Journal:  J Inherit Metab Dis       Date:  1989       Impact factor: 4.982

7.  New variant of hyperphenylalaninaemia with excretion of 7-substituted pterins.

Authors:  N Blau; J L Dhondt; P Guibaud; T Kuster; H C Curtius
Journal:  Eur J Pediatr       Date:  1988-11       Impact factor: 3.183

8.  Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism.

Authors:  B A Citron; S Kaufman; S Milstien; E W Naylor; C L Greene; M D Davis
Journal:  Am J Hum Genet       Date:  1993-09       Impact factor: 11.025

9.  Studies on the partially uncoupled oxidation of tetrahydropterins by phenylalanine hydroxylase.

Authors:  M D Davis; S Kaufman
Journal:  Neurochem Res       Date:  1991-07       Impact factor: 3.996

10.  Conversion of 6-substituted tetrahydropterins to 7-isomers via phenylalanine hydroxylase-generated intermediates.

Authors:  M D Davis; S Kaufman; S Milstien
Journal:  Proc Natl Acad Sci U S A       Date:  1991-01-15       Impact factor: 11.205
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