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A Model of Human Phenylalanine Metabolism in Normal Subjects and in Phenylketonuric Patients

Seymour Kaufman
Proceedings of the National Academy of Sciences of the United States of America
Vol. 96, No. 6 (Mar. 16, 1999), pp. 3160-3164
Stable URL: http://www.jstor.org/stable/47506
Page Count: 5
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Since scans are not currently available to screen readers, please contact JSTOR User Support for access. We'll provide a PDF copy for your screen reader.
A Model of Human Phenylalanine Metabolism in Normal Subjects and in Phenylketonuric Patients
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Abstract

The derivation of a quantitative model of phenylalanine metabolism in humans is described. The model is based on the kinetic properties of pure recombinant human phenylalanine hydroxylase and on estimates of the in vivo rates of phenylalanine transamination and protein degradation. Calculated values for the steady-state concentration of blood phenylalanine, rate of clearance of phenylalanine from the blood after an oral load of the amino acid, and dietary tolerance of phenylalanine all agree well with data from normal as well as from phenylketonuric patients and obligate heterozygotes. These calculated values may help in the decision about the degree of restriction of phenylalanine intake that is necessary to achieve a satisfactory clinical outcome in classical patients and in those with milder forms of the disease.

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