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Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria

In Phenylketonuria (PKU), the peptide structure of the protein substitute (PS), casein glycomacropeptide (CGMP), is supplemented with amino acids (CGMP-AA). CGMP may slow the rate of amino acid (AA) absorption compared with traditional phenylalanine-free amino acids (Phe-free AA), which may improve...

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Autores principales: Daly, Anne, Evans, Sharon, Pinto, Alex, Jackson, Richard, Ashmore, Catherine, Rocha, Júlio César, MacDonald, Anita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7468934/
https://www.ncbi.nlm.nih.gov/pubmed/32823853
http://dx.doi.org/10.3390/nu12082443
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author Daly, Anne
Evans, Sharon
Pinto, Alex
Jackson, Richard
Ashmore, Catherine
Rocha, Júlio César
MacDonald, Anita
author_facet Daly, Anne
Evans, Sharon
Pinto, Alex
Jackson, Richard
Ashmore, Catherine
Rocha, Júlio César
MacDonald, Anita
author_sort Daly, Anne
collection PubMed
description In Phenylketonuria (PKU), the peptide structure of the protein substitute (PS), casein glycomacropeptide (CGMP), is supplemented with amino acids (CGMP-AA). CGMP may slow the rate of amino acid (AA) absorption compared with traditional phenylalanine-free amino acids (Phe-free AA), which may improve nitrogen utilization, decrease urea production, and alter insulin response. Aim: In children with PKU, to compare pre and postprandial AA concentrations when taking one of three PS’s: Phe-free AA, CGMP-AA 1 or 2. Methods: 43 children (24 boys, 19 girls), median age 9 years (range 5–16 years) were studied; 11 took CGMP-AA1, 18 CGMP-AA2, and 14 Phe-free AA. Early morning fasting pre and 2 h postprandial blood samples were collected for quantitative AA on one occasion. A breakfast with allocated 20 g protein equivalent from PS was given post fasting blood sample. Results: There was a significant increase in postprandial AA for all individual AAs with all three PS. Postprandial AA histidine (p < 0.001), leucine (p < 0.001), and tyrosine (p < 0.001) were higher in CGMP-AA2 than CGMP-AA1, and leucine (p < 0.001), threonine (p < 0.001), and tyrosine (p = 0.003) higher in GCMP-AA2 than Phe-free AA. This was reflective of the AA composition of the three different PS’s. Conclusions: In PKU, the AA composition of CGMP-AA influences 2 h postprandial AA composition, suggesting that a PS derived from CGMP-AA may be absorbed similarly to Phe-free AA, but this requires further investigation.
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spelling pubmed-74689342020-09-04 Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria Daly, Anne Evans, Sharon Pinto, Alex Jackson, Richard Ashmore, Catherine Rocha, Júlio César MacDonald, Anita Nutrients Article In Phenylketonuria (PKU), the peptide structure of the protein substitute (PS), casein glycomacropeptide (CGMP), is supplemented with amino acids (CGMP-AA). CGMP may slow the rate of amino acid (AA) absorption compared with traditional phenylalanine-free amino acids (Phe-free AA), which may improve nitrogen utilization, decrease urea production, and alter insulin response. Aim: In children with PKU, to compare pre and postprandial AA concentrations when taking one of three PS’s: Phe-free AA, CGMP-AA 1 or 2. Methods: 43 children (24 boys, 19 girls), median age 9 years (range 5–16 years) were studied; 11 took CGMP-AA1, 18 CGMP-AA2, and 14 Phe-free AA. Early morning fasting pre and 2 h postprandial blood samples were collected for quantitative AA on one occasion. A breakfast with allocated 20 g protein equivalent from PS was given post fasting blood sample. Results: There was a significant increase in postprandial AA for all individual AAs with all three PS. Postprandial AA histidine (p < 0.001), leucine (p < 0.001), and tyrosine (p < 0.001) were higher in CGMP-AA2 than CGMP-AA1, and leucine (p < 0.001), threonine (p < 0.001), and tyrosine (p = 0.003) higher in GCMP-AA2 than Phe-free AA. This was reflective of the AA composition of the three different PS’s. Conclusions: In PKU, the AA composition of CGMP-AA influences 2 h postprandial AA composition, suggesting that a PS derived from CGMP-AA may be absorbed similarly to Phe-free AA, but this requires further investigation. MDPI 2020-08-14 /pmc/articles/PMC7468934/ /pubmed/32823853 http://dx.doi.org/10.3390/nu12082443 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Daly, Anne
Evans, Sharon
Pinto, Alex
Jackson, Richard
Ashmore, Catherine
Rocha, Júlio César
MacDonald, Anita
Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria
title Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria
title_full Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria
title_fullStr Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria
title_full_unstemmed Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria
title_short Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria
title_sort preliminary investigation to review if a glycomacropeptide compared to l-amino acid protein substitute alters the pre- and postprandial amino acid profile in children with phenylketonuria
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7468934/
https://www.ncbi.nlm.nih.gov/pubmed/32823853
http://dx.doi.org/10.3390/nu12082443
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