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The effect of the nitrogen source on metabolism in Phenylketonuria

Investigador: Maria João Pena, PhD Student, FMUPorto  
Instituição: Faculdade de Medicina da Universidade do Porto, Porto



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.
The main objective of the present research is to understand the effect of different nitrogen sources used for the treatment of PKU on metabolism.

In this project, we will test the hypothesis that GMP intake has different metabolic effects when compared to AAM in PKU context. For this, the following objectives were established:
1) To investigate if glycidic metabolism is affected by a diet containing GMP compared to a diet based on AAM in non-disease rat model in an acute way (Task 1);
2) To investigate if glycidic and lipid metabolism and gut microbiota are affected by a diet containing GMP compared to a diet based on AAM in non-disease rat model in a chronic way (Task 2);
3) To compare the gut microbiota of PKU patients with different treatments (Task 3).​





Faculdade de Farmácia da U.L.
Av. Prof. Gama Pinto
1649-003 Lisboa

Contacto: Fernanda Asper
Telefone.: +351 217 946 400
Fax: +351 217 946 491


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