MADISON Individuals with a rare genetic condition known as phenylketonuria, or PKU, receive a difficult-to-follow prescription. They must severely limit their consumption of protein, completely avoiding mealtime staples such as meat, cheese and even bread. Not surprisingly, for many, diet is a constant struggle.
In an effort to expand their dietary options, a team of University of Wisconsin-Madison scientists is assessing a unique protein found only in whey, the liquid byproduct of cheese-making, that appears to be safe for this group to eat. As published today in the Journal of Nutrition, the team found that this proteinwhen supplemented with a few amino acidssupports the growth of young, normal mice. More importantly, mice with a version of PKU fared well on this whey protein diet and had relatively low levels of PKU-associated toxins in their brain tissue and blood.
These findings open the door to the possibility that individuals with PKU will be able to eat foods enriched with the whey protein.
We did these studies in mice to show that (eating this protein) is going to be feasible in humans, says Denise Ney, a professor of nutritional sciences who headed up the study, which was funded by the UW-Madison Graduate School and the U.S. Department of Agriculture. Its so important to individuals on this diet to have new options, to have their diet liberalized. Its a quality-of-life issue.
There are around 15,000 people in the United States with PKU. From birth, they lack the enzyme responsible for breaking down phenylalanine, one of the 20 major amino acids that form the proteins we eat in everyday foods. They must avoid protein because, while small amounts of phenylalanine are required for life, excess amounts stay in their bodies indefinitely and interfere with brain function. Those who go off-diet often suffer from concentration problems and depression. A few even sustain permanent brain damage.
Instead of eating p
|Contact: Denise Ney|
University of Wisconsin-Madison