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The effect of the nitrogen source on metabolism in Phenylketonuria Investigador: Maria João Pena, PhD Student, FMUPorto Since Phenylketonuria’s (PKU) treatment introduction in 1950’s by Dr. Horst Bickel, many advances were made. In fact, one of the major contributions to PKU was the implementation of a phenylalanine (Phe)-restricted diet, remaining the cornerstone of treatment. The Phe-restricted diet consists of natural protein supplemented with protein substitutes (PS). Phe-free amino acid mixtures (AAM) are still the most common PS used to treat patients with PKU, despite the recent emergence of glycomacropeptide-based PS (GMP). GMP is an intact protein derived from cheese making process. There may be metabolic discrepancies between AAM and GMP, particularly at gycidic metabolism level. Several mechanisms can explain these differences, such as, the incretins release and the gut microbiota. Nevertheless, the scientific evidence is still scarce. This project comprises animal and clinical studies. |
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The effect of the nitrogen source on metabolism in Phenylketonuria Investigador: Maria João Pena, PhD Student, FMUPorto |
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