The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model
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| Název: | The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model |
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| Autoři: | Ahring, Kirsten K., Dagnæs-Hansen, Frederik, Brüel, Annemarie, Christensen, Mette, Jensen, Erik, Jensen, Thomas G., Johannsen, Mogens, Johansen, Karen S., Lund, Allan M., Madsen, Jesper G., Brøndum-Nielsen, Karen, Pedersen, Michael, Sørensen, Lambert K., Kjolby, Mads, Møller, Lisbeth B. |
| Zdroj: | Ahring, K K, Dagnæs-Hansen, F, Brüel, A, Christensen, M, Jensen, E, Jensen, T G, Johannsen, M, Johansen, K S, Lund, A M, Madsen, J G, Brøndum-Nielsen, K, Pedersen, M, Sørensen, L K, Kjolby, M & Møller, L B 2022, 'The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model', PLOS ONE, vol. 17, no. 1, e0261150. https://doi.org/10.1371/journal.pone.0261150 |
| Rok vydání: | 2022 |
| Sbírka: | Aarhus University: Research |
| Témata: | Amino Acids/blood, Animals, Bone Density, Bone and Bones/diagnostic imaging, Brain/metabolism, Caseins/therapeutic use, Dietary Supplements, Disease Models, Animal, Female, Maze Learning, Mice, Inbred C57BL, Peptide Fragments/therapeutic use, Phenylalanine Hydroxylase/deficiency, Phenylalanine/analysis, Phenylketonurias/diet therapy, Serotonin/blood, Tyrosine/blood |
| Popis: | Introduction Management of phenylketonuria (PKU) is mainly achieved through dietary control with limited intake of phenylalanine (Phe) from food, supplemented with low protein (LP) food and a mixture of free synthetic (FS) amino acids (AA) (FSAA). Casein glycomacropeptide (CGMP) is a natural peptide released in whey during cheese making by the action of the enzyme chymosin. Because CGMP in its pure form does not contain Phe, it is nutritionally suitable as a supplement in the diet for PKU when enriched with specific AAs. Lacprodan ® CGMP-20 (= CGMP) used in this study contained only trace amounts of Phe due to minor presence of other proteins/peptides. Objective The aims were to address the following questions in a classical PKU mouse model: Study 1, off diet: Can pure CGMP or CGMP supplemented with Large Neutral Amino Acids (LNAA) as a supplement to normal diet significantly lower the content of Phe in the brain compared to a control group on normal diet, and does supplementation of selected LNAA results in significant lower brain Phe level?. Study 2, on diet: Does a combination of CGMP, essential (non-Phe) EAAs and LP diet, provide similar plasma and brain Phe levels, growth and behavioral skills as a formula which alone consist of FSAA, with a similar composition?. Material and methods 45 female mice homozygous for the Pah enu2 mutation were treated for 12 weeks in five different groups; G1(N-CGMP), fed on Normal (N) casein diet (75%) in combination with CGMP (25%); G2 (N-CGMP-LNAA), fed on Normal (N) casein diet (75%) in combination with CGMP (19,7%) and selected LNAA (5,3% Leu, Tyr and Trp); G3 (N), fed on normal casein diet (100%); G4 (CGMP-EAA-LP), fed on CGMP (70,4%) in combination with essential AA (19,6%) and LP diet; G5 (FSAA-LP), fed on FSAA (100%) and LP diet. The following parameters were measured during the treatment period: Plasma AA profiles including Phe and Tyr, growth, food and water intake and number of teeth cut. At the end of the treatment period, a body scan (fat and lean body mass) and a ... |
| Druh dokumentu: | article in journal/newspaper |
| Jazyk: | English |
| DOI: | 10.1371/journal.pone.0261150 |
| Dostupnost: | https://pure.au.dk/portal/en/publications/e3729999-5996-45fb-b7e6-58ac73919dab https://doi.org/10.1371/journal.pone.0261150 http://www.scopus.com/inward/record.url?scp=85122635820&partnerID=8YFLogxK |
| Rights: | info:eu-repo/semantics/openAccess |
| Přístupové číslo: | edsbas.9C062579 |
| Databáze: | BASE |
Nájsť tento článok vo Web of Science | Abstrakt: | Introduction Management of phenylketonuria (PKU) is mainly achieved through dietary control with limited intake of phenylalanine (Phe) from food, supplemented with low protein (LP) food and a mixture of free synthetic (FS) amino acids (AA) (FSAA). Casein glycomacropeptide (CGMP) is a natural peptide released in whey during cheese making by the action of the enzyme chymosin. Because CGMP in its pure form does not contain Phe, it is nutritionally suitable as a supplement in the diet for PKU when enriched with specific AAs. Lacprodan ® CGMP-20 (= CGMP) used in this study contained only trace amounts of Phe due to minor presence of other proteins/peptides. Objective The aims were to address the following questions in a classical PKU mouse model: Study 1, off diet: Can pure CGMP or CGMP supplemented with Large Neutral Amino Acids (LNAA) as a supplement to normal diet significantly lower the content of Phe in the brain compared to a control group on normal diet, and does supplementation of selected LNAA results in significant lower brain Phe level?. Study 2, on diet: Does a combination of CGMP, essential (non-Phe) EAAs and LP diet, provide similar plasma and brain Phe levels, growth and behavioral skills as a formula which alone consist of FSAA, with a similar composition?. Material and methods 45 female mice homozygous for the Pah enu2 mutation were treated for 12 weeks in five different groups; G1(N-CGMP), fed on Normal (N) casein diet (75%) in combination with CGMP (25%); G2 (N-CGMP-LNAA), fed on Normal (N) casein diet (75%) in combination with CGMP (19,7%) and selected LNAA (5,3% Leu, Tyr and Trp); G3 (N), fed on normal casein diet (100%); G4 (CGMP-EAA-LP), fed on CGMP (70,4%) in combination with essential AA (19,6%) and LP diet; G5 (FSAA-LP), fed on FSAA (100%) and LP diet. The following parameters were measured during the treatment period: Plasma AA profiles including Phe and Tyr, growth, food and water intake and number of teeth cut. At the end of the treatment period, a body scan (fat and lean body mass) and a ... |
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| DOI: | 10.1371/journal.pone.0261150 |