Document Detail

Purification and use of glycomacropeptide for nutritional management of phenylketonuria.
MedLine Citation:
PMID:  19490325     Owner:  NLM     Status:  MEDLINE    
Individuals with phenylketonuria (PKU) cannot metabolize phenylalanine (Phe) and must adhere to a low-Phe diet in which most dietary protein is provided by a Phe-free amino acid formula. Glycomacropeptide (GMP) is the only naturally occurring protein that does not contain Phe, and is of interest as a source of protein for dietary management of PKU. However, commercially available GMP contains too much Phe from residual whey proteins and does not contain adequate levels of all the indispensable amino acids to provide a nutritionally complete protein. The aim of this study was to increase purity of GMP and develop a mass balance calculation for indispensable amino acid supplementation of GMP foods. Cation exchange chromatography, ultrafiltration/diafiltration, and lyophilization were used at the pilot plant scale to decrease Phe. Enough purified GMP (5 kg) was manufactured to provide 15 PKU subjects with a 4-d diet in which the majority of protein was from GMP foods. A mass balance was used to supplement GMP foods so that all indispensable amino acids met or exceeded the daily recommended intake. GMP foods were tested in a human clinical trial as a replacement for the traditional amino acid formula. Nutritionally complete GMP foods created with high purity GMP provide individuals with PKU with more options to manage PKU, which may lead to improved compliance and quality of life.
Caitlin E Laclair; Denise M Ney; Erin L MacLeod; Mark R Etzel
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of food science     Volume:  74     ISSN:  1750-3841     ISO Abbreviation:  J. Food Sci.     Publication Date:    2009 May-Jul
Date Detail:
Created Date:  2009-06-03     Completed Date:  2009-09-01     Revised Date:  2013-06-02    
Medline Journal Info:
Nlm Unique ID:  0014052     Medline TA:  J Food Sci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  E199-206     Citation Subset:  IM    
Dept. of Food Science, Univ. of Wisconsin, Madison, WI 53706, USA.
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MeSH Terms
Amino Acids / administration & dosage
Caseins / administration & dosage*,  chemistry,  isolation & purification*
Chromatography, Ion Exchange
Freeze Drying
Lysine / administration & dosage
Milk / chemistry
Nutritional Requirements
Peptide Fragments / administration & dosage*,  chemistry,  isolation & purification*
Phenylalanine / analysis
Phenylketonurias / diet therapy*
Grant Support
Reg. No./Substance:
0/Amino Acids; 0/Caseins; 0/Peptide Fragments; 0/caseinomacropeptide; 56-87-1/Lysine; 63-91-2/Phenylalanine

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