Metabolomic Markers of Essential Fatty Acids, Carnitine, and Cholesterol Metabolism in Adults and Adolescents with Phenylketonuria

The Journal of Nutrition
Bridget M StroupDenise M Ney

Abstract

Individuals with phenylketonuria (PKU) have a risk of cognitive impairment and inflammation. Many follow a low-phenylalanine (low-Phe) diet devoid of animal protein in combination with medical foods (MFs). To assess lipid metabolism in participants with PKU consuming amino acid MFs (AA-MFs) or glycomacropeptide MFs (GMP-MFs), we conducted fatty acid and metabolomics analyses. We used subsets of fasting plasma and urine samples from our randomized crossover trial in which participants with early-treated classical and variant (milder) PKU consumed a low-Phe diet combined with AA-MFs or GMP-MFs for 3 wk each. Fatty acid profiles of red blood cell (RBC) membranes were determined for 25 adults (aged 18-49 y) with PKU and 143 control participants. Metabolomics analyses of plasma and urine samples were conducted by Metabolon for 9-10 adolescent and adult participants with PKU and for 15 control participants. RBC fatty acid profiles were not significantly different with AA-MFs or GMP-MFs. PKU participants showed higher total n-6:n-3 (ω-6:ω-3) fatty acids (mean ± SD percentages of total fatty acids: AA-MF = 5.45% ± 1.07%; controls = 4.33%; P < 0.001) and lower docosahexaenoic acid (DHA; AA-MF = 3.21% ± 0.98%; controls = 3.70% ± 1.01%; P...Continue Reading

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Citations

Mar 7, 2020·Journal of Neuroscience Research·Bruna Klippel FerreiraPatricia Fernanda Schuck
Oct 26, 2018·Nutritional Neuroscience·Penelope D Manta-VogliKleopatra H Schulpis
Sep 29, 2020·Frontiers in Endocrinology·Els van der GootEddy A van der Zee
May 29, 2020·Archives of Biochemistry and Biophysics·Inês M S GuerraAna S P Moreira
Apr 6, 2021·Journal of Proteome Research·Inês M S GuerraAna S P Moreira
Dec 6, 2021·The American Journal of Clinical Nutrition·Meriah S Schoen, Rani H Singh

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