Rate of Phenylalanine Hydroxylation in Healthy School-Aged Children

Abstract

Hydroxylation of phenylalanine to tyrosine is the first and rate-limiting step in phenylalanine catabolism. Currently, there are data on the rate of phenylalanine hydroxylation in infants and adults but not in healthy children. Thus, the aim of the study reported here was to measure the rate of phenylalanine hydroxylation and oxidation in healthy school-aged children both when receiving diets with and without tyrosine. In addition, hydroxylation rates calculated from the isotopic enrichments of amino acids in plasma and in very LDL apoB-100 were compared. Eight healthy 6- to 10-y-old children were studied while receiving a control and again while receiving a tyrosine-free diet. Phenylalanine flux, hydroxylation, and oxidation were determined by a standard tracer protocol using oral administration of ¹³C-phenylalanine and ²H₂-tyrosine for 6 h. Phenylalanine hydroxylation rate of children fed a diet devoid of tyrosine was greater than that of children fed a diet containing tyrosine (40.25 ± 5.48 versus 29.55 ± 5.35 μmol · kg⁻¹ · h⁻¹; p < 0.01). Phenylalanine oxidation was not different from phenylalanine hydroxylation regardless of dietary tyrosine intake, suggesting that phenylalanine converted to tyrosine was mainly oxidized. In conclusion, healthy children are capable of converting phenylalanine to tyrosine, but the need for tyrosine cannot be met by providing extra phenylalanine.