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

Pediatric Research
|December 24, 2010
PubMed

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.

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