A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria.
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.
Langenbeck
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U
Institute of Human Genetics, University of Göttingen, Germany.
Behbehani
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Mench-Hoinowski
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D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007668:Kidney / Q000378:metabolism; D007773:Lactates / Q000378:metabolism; D008297:Male; D010648:Phenylacetates / Q000378:metabolism; D010649:Phenylalanine / Q000378:metabolism*; D010661:Phenylketonurias / Q000209:etiology / Q000378:metabolism*; D010667:Phenylpyruvic Acids / Q000378:metabolism
D007773:Lactates; D010648:Phenylacetates; D010667:Phenylpyruvic Acids; D010649:Phenylalanine; C005434:atrolactic acid; C005756:2-hydroxyphenylacetic acid; C031606:phenylpyruvic acid