Literature DB >> 1583868

A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria.

U Langenbeck1, A Behbehani, A Mench-Hoinowski.   

Abstract

We present blood and urine levels of unconjugated o-hydroxyphenylacetic, phenyllactic and phenylpyruvic acids in 61 children (2 years of age and above) and juveniles with phenylketonuria on or partially off diet. The samples were obtained during 185 scheduled outpatient visits and have been analysed with gas chromatographic methods. The compiled data define reference ranges of phenylalanine transamination capacity and of renal transport of metabolites which may be of value in further studies on the pathogenesis of phenylketonuria.

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Year:  1992        PMID： 1583868     DOI： 10.1007/bf01800355

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


  32 in total

1.  Plasma concentrations of phenyllactic acid in phenylketonuria.

Authors:  P C Clemens; M H Schünemann; G F Hoffmann; A Kohlschütter
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

Review 2.  The salience of Garrod's 'molecular groupings' and 'Inborn Factors in Disease'.

Authors:  C R Scriver
Journal:  J Inherit Metab Dis       Date:  1989       Impact factor: 4.982

3.  Phenylketonemia in phenylketonuria.

Authors:  M W Partington; S K Vickery
Journal:  Neuropadiatrie       Date:  1974-05

4.  Phenylalanine loading and aromatic acid excretion in normal subjects and heterozygotes for phenylketonuria.

Authors:  K Blau; G K Summer; H C Newsome; C H Edwards
Journal:  Clin Chim Acta       Date:  1973-05-18       Impact factor: 3.786

5.  An improved method for measuring blood concentrations of phenylpyruvic acid.

Authors:  S P Coburn; J D Mahuren; R W Fuller
Journal:  Clin Chem       Date:  1971-05       Impact factor: 8.327

6.  Acidic metabolites of phenylalanine in plasma of phenylketonurics.

Authors:  M Tuchman; R O Fisch; M L Ramnaraine; W Krivit
Journal:  Biochem Med       Date:  1985-10

7.  O-trimethylsilylquinoxalinol derivatives of aromatic alpha-keto acids. Mass spectra and quantitative gas chromatography.

Authors:  U Langenbeck; A Mench-Hoinowski; K P Dieckmann; H U Möhring; M Petersen
Journal:  J Chromatogr       Date:  1978-03-01

8.  Phenylalanine metabolites, attention span and hyperactivity.

Authors:  K Michals; R Matalon
Journal:  Am J Clin Nutr       Date:  1985-08       Impact factor: 7.045

9.  Quantitative analysis of beta-phenylpyruvic acid by single ion monitoring. Evaluation of isomeric internal standards.

Authors:  U Langenbeck; A Mench-Hoinowski; I Rød-Urban
Journal:  J Chromatogr       Date:  1978-09-01

10.  Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man.

Authors:  W Krause; M Halminski; L McDonald; P Dembure; R Salvo; D Freides; L Elsas
Journal:  J Clin Invest       Date:  1985-01       Impact factor: 14.808
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  5 in total

1.  Development of Metabolic Phenotype in Phenylketonuria: Evaluation of the Blaskovics Protein Loading Test at 5 Years of Age.

Authors:  P Burgard; E Mönch; J Zschocke; U Wendel; U Langenbeck
Journal:  JIMD Rep       Date:  2015-12-19

2.  Modelling the phenylalanine blood level response during treatment of phenylketonuria.

Authors:  U Langenbeck; J Zschocke; U Wendel; V Hönig
Journal:  J Inherit Metab Dis       Date:  2001-12       Impact factor: 4.982

3.  In vivo disposal of phenylalanine in phenylketonuria: a study of two siblings.

Authors:  E Treacy; J J Pitt; K Seller; G N Thompson; S Ramus; R G Cotton
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982

4.  A model of human phenylalanine metabolism in normal subjects and in phenylketonuric patients.

Authors:  S Kaufman
Journal:  Proc Natl Acad Sci U S A       Date:  1999-03-16       Impact factor: 11.205

5.  Effect of phenylalanine and its metabolites on ATP diphosphohydrolase activity in synaptosomes from rat cerebral cortex.

Authors:  A T Wyse; J J Sarkis; J S Cunha-Filho; M V Teixeira; M R Schetinger; M Wajner; C Milton; D Wannmacher
Journal:  Neurochem Res       Date:  1994-09       Impact factor: 3.996
  5 in total

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