| Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria. | |
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MedLine Citation:
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PMID: 22297302 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Phenylketonuria (PKU) is caused by a mutation in the phenylalanine (phe) hydroxylase gene and requires a low-phe diet plus amino acid (AA) formula to prevent cognitive impairment. Glycomacropeptide (GMP) contains minimal phe and provides a palatable alternative to AA formula. Our objective was to compare growth, body composition, and energy balance in Pah(enu2) (PKU) and wild type (WT) mice fed low-phe GMP, low-phe AA or high-phe casein diets from 3-23 wks of age. The 2x2x3 design included main effects of genotype, sex and diet. Fat and lean mass were assessed by dual-energy x-ray absorptiometry and acute energy balance was assessed by indirect calorimetry. PKU mice showed growth and lean mass similar to WT littermates fed the GMP or AA diets; however, they exhibited a 3-15% increase in energy expenditure, as reflected in oxygen consumption, and a 3-30% increase in food intake. The GMP diet significantly reduced energy expenditure, food intake and plasma phe concentration in PKU mice compared with the casein diet. The high-phe casein diet or the low-phe AA diet induced metabolic stress in PKU mice as reflected in increased energy expenditure and intake of food and water, increased renal and spleen mass, and elevated plasma cytokine concentrations consistent with systemic inflammation. The low-phe GMP diet significantly attenuated these adverse effects. Moreover, total fat mass, % body fat and the respiratory exchange ratio (CO(2) produced /O(2) consumed) were significantly lower in PKU mice fed GMP compared with AA diets. In summary, GMP provides a physiologic source of low-phe dietary protein that promotes growth and attenuates the metabolic stress induced by a high-phe casein or low-phe AA diet in PKU mice. |
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Authors:
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Patrick M Solverson; Sangita G Murali; Adam S Brinkman; David W Nelson; Murray K Clayton; Chi-Liang Eric Yen; Denise M Ney |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2012-1-31 |
Journal Detail:
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Title: American journal of physiology. Endocrinology and metabolism Volume: - ISSN: 1522-1555 ISO Abbreviation: - Publication Date: 2012 Jan |
Date Detail:
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Created Date: 2012-2-2 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100901226 Medline TA: Am J Physiol Endocrinol Metab Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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1University of Wisconsin-Madison. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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