PMID: 18203898Jan 22, 2008Paper

Dietary glycomacropeptide supports growth and reduces the concentrations of phenylalanine in plasma and brain in a murine model of phenylketonuria

The Journal of Nutrition
Denise M NeyMark R Etzel

Abstract

Phenylketonuria (PKU) is a genetic disorder caused by deficiency of phenylalanine hydroxylase (PAH) that requires life-long adherence to a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP) is uniquely suited to the nutritional management of PKU, because pure GMP contains no Phe. Our aim was to assess how ingestion of diets containing GMP support growth and affect the concentrations of amino acids in plasma and brains of mice with a deficiency of PAH, the Pah(enu2) mouse (PKU mouse). Experiments were conducted in 4- to 6-wk-old wild-type (WT) (C57Bl/6) and PKU mice fed diets containing 20% protein from casein, amino acids, or GMP supplemented with limiting indispensable amino acids (IAA). PKU mice fed the GMP diet showed gains in body weight, feed efficiency, and a protein efficiency ratio that did not differ from the amino acid diet. The concentrations of isoleucine and threonine in plasma showed a significant 2- to 3-fold increase for WT and PKU mice fed GMP compared with casein or amino acid diets, respectively. PKU mice fed the GMP diet had decreased concentrations of Phe in plasma (11% decrease) and in 5 regions of the brain (20% decrease) compared with the amino acid diet. The concentration of Phe in the brain was inve...Continue Reading

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Citations

Feb 27, 2009·The American Journal of Clinical Nutrition·Sandra C van CalcarDenise M Ney
Oct 29, 2008·Journal of Inherited Metabolic Disease·D M NeyJ A Wolff
Apr 13, 2017·European Journal of Clinical Nutrition·A PintoJ C Rocha
Jun 18, 2016·Journal of Nutrition and Metabolism·Osama K ZakiAhmed Elghawaby
Apr 28, 2016·European Journal of Clinical Nutrition·M J PenaJ C Rocha
Oct 26, 2018·Nutritional Neuroscience·Penelope D Manta-VogliKleopatra H Schulpis
Jun 6, 2009·Journal of Food Science·Caitlin E LaclairMark R Etzel
Jul 6, 2010·The Proceedings of the Nutrition Society·Fermín Sánchez de MedinaOlga Martínez-Augustin
Jan 24, 2017·Journal of Human Nutrition and Dietetics : the Official Journal of the British Dietetic Association·A DalyA MacDonald
Feb 6, 2021·Nutrients·Anne DalyAnita MacDonald
Feb 7, 2012·Nutrition & Metabolism·Marcello GiovanniniEnrica Riva

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