Phenylketonuria (PKU)

Phenylketonuria (PKU)

Phenylketonuria (PKU)

Improved PKU nutrition with Lacprodan® CGMP-20

PKU is a rare metabolic disorder that disrupts the metabolism of the amino acid phenylalanine. If not treated, the level of phenylalanine builds up in the blood and poisons nerve cells (neurons) in the brain, causing brain damage and intellectual disability. 

There is no cure for PKU, but treatment can prevent intellectual disabilities and other health problems. People with PKU need to follow a special dietary regime that limits foods with phenylalanine. The diet should be followed carefully and be started as soon after birth as possible.

The current PKU diet consists of a low protein diet and free amino acid supplements in order to limit the patient's intake of phenylalanine.

Lacprodan® CGMP-20  is a highly purified casein glycomacropeptide (CGMP) product that is an ideal protein source for PKU patients since it is very low in the amino acid phenylalanine. Lacprodan® CGMP-20 can substitute the main part of the free amino acids in the PKU diet and provide several beneficial nutritional effects compared to free amino acids.

Lacprodan® CGMP-20 contains a high amounts of large neutral amino acids, such as threonine and isoleucine, which compete for uptake into the brain with phenylalanine1, and potentially, preventing accumulation of phenylalanine in the brain.

The fact that Lacprodan® CGMP-20 is a polypeptide is associated with a more beneficial absorption rate and body retention, compared to free amino acids2. Additionally, the absorption rate of CGMP has been linked to more stable blood phenylalanine levels over 24 hours. The maintenance of phenylalanine levels in the desired target range may have positive effect for patients with PKU3. Moreover, Lacprodan® CGMP-20 is a sialic acid-containing polypeptide with potential benefits for the gastrointestinal tract4.

Another important benefit of Lacprodan® CGMP-20 is that the taste is significantly improved when it substitutes part of the free amino acids, and this may help ensure high patient compliance with the PKU diet4.

From an application perspective, Lacprodan® CGMP-20 is a versatile, palatable ingredient with a high purity, that can be used in UHT drinks, bars and powders.

 

References:

  1. Larsen, E.R., et al., Dietary supplementation with casein glycomacropeptide, leucine and tryptophan reduces plasma amino acid levels in men. Acta Neuropsychiatr, 2021: p. 1-8.
  2. MacDonald, A., et al., PKU dietary handbook to accompany PKU guidelines. Orphanet J Rare Dis, 2020. 15(1): p. 171.
  3. Daly, A., et al., The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trial. Nutrients, 2019. 11(3).
  4. Daly, A., et al., Glycomacropeptide in PKU-Does It Live Up to Its Potential? Nutrients, 2022. 14(4).
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