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Published on: April 22, 2022
Rate of phenylalanine hydroxylation in healthy school-aged children
Jean W Hsu1, Farook Jahoor, Nancy F Butte
1Department of Pediatrics, USDA-ARS Children's Nutrition Research Center, Baylor College of Medicine, Houston, Texas 77030, USA. wheird@bcm.tmc.edu
Insights
Healthy children convert phenylalanine to tyrosine, a key step in metabolism. This study found that a tyrosine-free diet significantly increased phenylalanine hydroxylation rates in school-aged children.
Area of Science:
- Biochemistry
- Human Physiology
- Nutritional Science
Background:
- Phenylalanine to tyrosine conversion is crucial for phenylalanine metabolism.
- Data on phenylalanine hydroxylation rates are lacking in healthy children.
Purpose of the Study:
- To measure phenylalanine hydroxylation and oxidation rates in healthy school-aged children.
- To compare these rates with and without dietary tyrosine.
- To compare hydroxylation rates calculated from plasma and very low-density lipoprotein (VLDL) apolipoprotein B-100 (apoB-100) isotopic enrichments.
Main Methods:
- Eight healthy children (6-10 years old) participated in a controlled study.
- Children received both a control diet and a tyrosine-free diet.
- Phenylalanine flux, hydroxylation, and oxidation were measured using a 6-hour tracer protocol with ¹³C-phenylalanine and ²H₂-tyrosine.
Main Results:
- Phenylalanine hydroxylation rate was significantly higher in children on a tyrosine-free diet (40.25 ± 5.48 μmol·kg⁻¹·h⁻¹) compared to a control diet (29.55 ± 5.35 μmol·kg⁻¹·h⁻¹).
- Phenylalanine oxidation rates were similar to hydroxylation rates, indicating efficient conversion and subsequent oxidation.
- Dietary tyrosine availability influences phenylalanine hydroxylation rate in children.
Conclusions:
- Healthy children can convert phenylalanine to tyrosine.
- The body's tyrosine requirements cannot be solely met by increasing phenylalanine intake.
- Findings highlight the importance of dietary tyrosine for maintaining normal phenylalanine metabolism in 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.
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