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A Model of Human Phenylalanine Metabolism in Normal Subjects and in Phenylketonuric Patients
Proceedings of the National Academy of Sciences of the United States of America
Vol. 96, No. 6 (Mar. 16, 1999), pp. 3160-3164
Published by: National Academy of Sciences
Stable URL: http://www.jstor.org/stable/47506
Page Count: 5
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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.
Proceedings of the National Academy of Sciences of the United States of America © 1999 National Academy of Sciences