Abstract
Renal clearance of phenylpyruvic acid is maximal at a plasma concentration of 40–60 µmol/l. This concentration is obtained with plasma phenylalanine concentrations of 1.0–1.2mmol/l, the threshold for separating classical phenylketonuria from phenylketonuria variants.
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Langenbeck, U. Mental retardation and inborn errors of metabolism.Lancet 1 (1973) 785
Langenbeck, U. Quantitative gas chromatography of α-keto acids with a nitrogen-selective detector.Fresenius Z. Anal. Chem. 290 (1978) 132–133
Langenbeck, U., Mench-Hoinoswki, A., Dieckmann, K.-P., Möhring, H.-U. and Petersen, M.O-Trimethylsilylquinoxalinol derivatives of aromatic α-keto acids. Mass spectra and quantitative gas chromatography.J. Chromatogr. 145 (1978a) 185–193
Langenbeck, U., Mench-Hoinowski, A. and Rød-Urban, I. Quantitative analysis of β-phenylpyruvic acid by single ion monitoring. Evaluation of isomeric internal standards.J. Chromatogr. 146 (1978b) 213–219
Langenbeck, U., Mench-Hoinowski, A., Rød-Urban, I. and Luthe, H. Quantitative determination of plasma phenylpyruvic acid by GC-MS with an isomeric internal standard.Quant. MS in Life Sci. II (1978c) 209–218
Langenbeck, U., Behbehani, A., Mench-Hoinowski, A. and Petersen, M. Absence of a significant renal threshold for two aromatic acids in phenylketonuric children over two years of age.Eur. J. Pediatr. 134 (1980a) 115–118
Langenbeck, U., Behbehani, A. and Luthe, H. Blood levels of phenylpyruvic acid in phenylketonuria.J. Clin. Chem. Clin. Biochem. 18 (1980b) 755
Sitzmann, F. C.Normalwerte, Hans Marseille Verlag, München, 1976, p. 171
Vink, C. L. J. and Kroes, A. A. The renal clearance of phenylpyruvate.Clin. Chim. Acta 6 (1961) 813–818
Zelniček, E. and Podkradská, O. Clearance of endogenous phenylpyruvate in phenylketonurics.Clin. Chim. Acta 25 (1969) 179–180
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Langenbeck, U., Behbehani, A. & Luthe, H. Renal transport of aromatic acids in patients with phenylketonuria. J Inherit Metab Dis 4, 69–70 (1981). https://doi.org/10.1007/BF02263595
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DOI: https://doi.org/10.1007/BF02263595