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

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Journal of Inherited Metabolic Disease

Summary

We present blood and urine levels of unconjugatedo-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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Authors and Affiliations

  1. Institute of Human Genetics, University of Göttingen, D-3400, Göttingen, Germany

    U. Langenbeck & A. Mench-Hoinowski

  2. formerly Children's Hospital, University of Göttingen, D-3400, Göttingen, Germany

    A. Behbehani

  3. Institute of Human Genetics, University Hospital, Theodor-Stern-Kai 7, D-6000, Frankfurt am Main 70, Germany

    U. Langenbeck

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  1. U. Langenbeck
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  2. A. Behbehani
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  3. A. Mench-Hoinowski
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Langenbeck, U., Behbehani, A. & Mench-Hoinowski, A. A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria. J Inherit Metab Dis 15, 136–144 (1992). https://doi.org/10.1007/BF01800355

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  • DOI: https://doi.org/10.1007/BF01800355

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