对于苯丙酮尿症患者，可使用奶酪乳清中的糖巨肽制作可接受的低苯丙氨酸食品和饮料。
Acceptable low-phenylalanine foods and beverages can be made with glycomacropeptide from cheese whey for individuals with PKU.
作者信息
Lim Kyungwha, van Calcar Sandra C, Nelson Kathryn L, Gleason Sally T, Ney Denise M
机构信息
Wisconsin Center for Dairy Research, University of Wisconsin, Madison, WI 53706, USA.
出版信息
Mol Genet Metab. 2007 Sep-Oct;92(1-2):176-8. doi: 10.1016/j.ymgme.2007.06.004. Epub 2007 Jul 23.
Abstract
Glycomacropeptide (GMP) is a whey protein that contains no aromatic amino acids including phenylalanine (phe). The objective of this study was to make a variety of palatable, low-phe foods and beverages with GMP and to assess their acceptability by conducting consumer sensory studies in individuals with PKU. Results demonstrate acceptability of products made with GMP. GMP supplemented with limiting indispensable amino acids could provide an alternative protein source for individuals with PKU.
摘要
糖巨肽（GMP）是一种乳清蛋白，不含包括苯丙氨酸（phe）在内的芳香族氨基酸。本研究的目的是用GMP制作各种可口的低苯丙氨酸食品和饮料，并通过对苯丙酮尿症患者进行消费者感官研究来评估其可接受性。结果表明了用GMP制作的产品的可接受性。补充了限制必需氨基酸的GMP可为苯丙酮尿症患者提供一种替代蛋白质来源。
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本文引用的文献
1
Manufacture and use of dairy protein fractions.
J Nutr. 2004 Apr;134(4):996S-1002S. doi: 10.1093/jn/134.4.996S.
2
Dietary reference intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids.
J Am Diet Assoc. 2002 Nov;102(11):1621-30. doi: 10.1016/s0002-8223(02)90346-9.
3
Phenylketonuria in adulthood: a collaborative study.
J Inherit Metab Dis. 2002 Sep;25(5):333-46. doi: 10.1023/a:1020158631102.
4
Phenylketonuria (PKU): screening and management.
NIH Consens Statement. 2000;17(3):1-33.
5
Biological activities of bovine glycomacropeptide.
Br J Nutr. 2000 Nov;84 Suppl 1:S39-46. doi: 10.1017/s0007114500002233.
6
Necessity of complete intake of phenylalanine-free amino acid mixture for metabolic control of phenylketonuria.
J Am Diet Assoc. 1999 Dec;99(12):1559-63. doi: 10.1016/S0002-8223(99)00382-X.
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