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Nutritional management of PKU with glycomacropeptide from cheese whey

  • BH4 and PKU
  • Published:
Journal of Inherited Metabolic Disease

Summary

Individuals with phenylketonuria (PKU) must follow a lifelong low-phenylalanine (Phe) diet to prevent neurological impairment. Compliance with the low-Phe diet is often poor owing to restriction in natural foods and the requirement for consumption of a Phe-free amino acid formula or medical food. Glycomacropeptide (GMP), a natural protein produced during cheese-making, is uniquely suited to a low-Phe diet because when isolated from cheese whey it contains minimal Phe (2.5–5 mg Phe/g protein). This paper reviews progress in evaluating the safety, acceptability and efficacy of GMP in the nutritional management of PKU. A variety of foods and beverages can be made with GMP to improve the taste, variety and convenience of the PKU diet. Sensory studies in individuals with PKU demonstrate that GMP foods are acceptable alternatives to amino acid medical foods. Studies in the PKU mouse model demonstrate that GMP supplemented with limiting indispensable amino acids provides a nutritionally adequate source of protein and improves the metabolic phenotype by reducing concentrations of Phe in plasma and brain. A case report in an adult with classical PKU who followed the GMP diet for 10 weeks at home indicates safety, acceptability of GMP food products, a 13–14% reduction in blood Phe levels (p < 0.05) and improved distribution of dietary protein throughout the day compared with the amino acid diet. In summary, food products made with GMP that is supplemented with limiting indispensable amino acids provide a palatable alternative source of protein that may improve dietary compliance and metabolic control of PKU.

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Abbreviations

AA:

amino acid

GMP:

glycomacropeptide

LNAA:

large neutral amino acids

MS/MS:

tandem mass spectroscopy

PAH:

phenylalanine hydroxylase

Phe:

phenylalanine

PKU:

phenylketonuria

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Acknowledgements

This work was supported by the Mid Atlantic Connection for PKU and Allied Disorders, Michaux Family Foundation, Robert Draper Technology Innovation Fund and the USDA Cooperative State Research, Education and Extension Service, project WISO1084, University of Wisconsin-Madison; and by NIH R03-DK-071534 and P30-HD-03352. In addition, the authors acknowledge the assistance of Phillip Williams, Waisman Center Biochemical Genetics Laboratory, for conducting the plasma amino acid analyses; Kimberlee J. Burrington, Coordinator, Dairy Ingredients Applications Group, Wisconsin Center for Dairy Research for her support in developing the GMP food products; and Xiaowen Liu for assistance with statistical analysis. We also acknowledge the GMP Task Force for initially recognizing the potential contribution of GMP to the nutritional management of PKU and facilitating this research. Members of the Task Force include two whey-producing companies, Davisco Foods of Minnesota and Grande Cheese Co. of Wisconsin, and two companies that distribute medical foods for the PKU market, Cambrooke Foods of Boston and Applied Nutrition of New Jersey. Membership also includes faculty and staff of the University of Wisconsin-Madison, parents of children with PKU, and an adult with PKU.

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Authors and Affiliations

  1. Department of Nutritional Sciences, University of Wisconsin, 1415 Linden Drive, Madison, WI, 53706, USA

    D. M. Ney, S. T. Gleason & E. L. MacLeod

  2. Waisman Center, University of Wisconsin, Madison, Wisconsin, USA

    S. C. van Calcar, G. M. Rice & J. A. Wolff

  3. Wisconsin Center for Dairy Research, University of Wisconsin, Madison, Wisconsin, USA

    K. L. Nelson

  4. Department of Food Science, University of Wisconsin, Madison, Wisconsin, USA

    M. R. Etzel

Authors
  1. D. M. Ney
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  2. S. T. Gleason
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  3. S. C. van Calcar
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  4. E. L. MacLeod
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  5. K. L. Nelson
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  6. M. R. Etzel
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  7. G. M. Rice
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  8. J. A. Wolff
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Corresponding author

Correspondence to D. M. Ney.

Additional information

Communicating editor: Beat Thöny

Competing interests: None declared

References to electronic databases: Phenylalanine hydroxylase: EC 1.14.16.1.

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Ney, D.M., Gleason, S.T., van Calcar, S.C. et al. Nutritional management of PKU with glycomacropeptide from cheese whey. J Inherit Metab Dis 32, 32–39 (2009). https://doi.org/10.1007/s10545-008-0952-4

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  • DOI: https://doi.org/10.1007/s10545-008-0952-4

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