By Erik Jensen, Research Scientist, Arla Foods Ingredients
Avoiding meat, dairy products and other protein rich foods? Being forced to drink unpleasant-tasting liquids or taking large amounts of tablets several times a day?
Those are just some of the challenges faced by people with phenylketonuria (PKU). It’s an inborn error of metabolism that affects around 1 in 10,000– 15,000 people.
Basically, PKU patients are not able to sufficiently metabolise the amino acid phenylalanine, which is naturally found in all protein types. If PKU patients follow a normal diet, it will result in accumulation of phenylalanine in the blood and brain to toxic levels. High levels of phenylalanine may result in brain damage and mental retardation in PKU infants and children, if left untreated.
To maintain phenylalanine levels in a healthy range, PKU patients are recommended a restrictive diet based on low-protein foods, combined with protein substitutes consisting of artificial, free amino acids devoid of phenylalanine. These free amino acid formulas often have an offensive taste and smell.
The PKU diet has quite an impact on patient quality of life. For example, the free amino acid formulas don’t just taste unpleasant, they can also result in bad breath, due to specific, sulfur-containing amino acids. And for many patients, the last thing they want to do is to stand out from the crowd by eating different food from their friends and colleagues and having to drink special supplements or take more than 30 grams of pills throughout the day.
That sort of thing isn’t very motivating – and many teenagers, in particular, once free of parental guidance, neglect their disease in the hunt for social acceptance. In adult life, some even quit their diet regimes completely. But such neglect, unfortunately, can lead to cognitive impairment, attention problems and mood disorders.
Several years ago, scientists discovered that a specific protein in whey is very suitable for PKU patients. The protein is called CGMP (Casein GlycoMacroPeptide) and is free from phenylalanine in its pure form.
However, it’s only recently that we have managed to produce CGMP on a commercial scale at a purity which allows it to deliver its full potential. This high purity is achieved by combining different production technologies that make it possible to efficiently remove other whey protein types containing phenylalanine.
When we discovered how to produce highly purified CGMP from bovine whey, we realised that we could transform the lifestyles of people with PKU, making life much more enjoyable. With CGMP replacing free amino acids, patients are now able to consume a good-tasting, natural protein that will help keep them on their diets. CGMP also has beneficial effects on satiety, gut and bone health, too.
We believe it is important to help build evidence for potential health benefits of CGMP in PKU patients, and we’re focused on supporting PKU research. We are, therefore, highly involved in setting up and sponsoring new studies within PKU nutrition.
The road ahead for CGMP looks very exciting, and I’ll keep you posted as this future unfolds.
This blog contains material and information intended for B2B customers, suppliers and distributors, and is not intended as information to the final consumers.