Journal of Nutrition and Metabolism / 2017 / Article / Fig 3

Clinical Study

Metabolomic Insights into the Nutritional Status of Adults and Adolescents with Phenylketonuria Consuming a Low-Phenylalanine Diet in Combination with Amino Acid and Glycomacropeptide Medical Foods

Figure 3

Urine metabolomics of sulfur-containing amino acid (Met and Cys) metabolism, n = 9. Values are means ± SE. Participants with PKU consumed significantly more Met and Cys from medical foods with AA-MF compared to GMP-MF, n = 8 (means ± SE; Met, g/d: AA-MF, 1.3 ± 0.2, versus GMP-MF, 0.9 ± 0.1, ; Cys, g/d: AA-MF, 2.5 ± 0.3, versus GMP-MF, 0.3 ± 0.007, ). Participants had higher urinary excretion of taurine and taurine-related metabolites (hypotaurine, cysteine sulfonate, cysteine, and cystathionine), possibly related to higher intakes of sulfur-containing amino acids and increased need to excrete sulfur. AA-MF, amino acid medical foods; GMP-MF, glycomacropeptide medical foods.

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