PKU

What is PKU?

Phenylketonuria, or PKU is an inborn error in metabolism. It is a defect in the enzyme  Phenylalnine hydroxylase, which normally processes the amino acid phenylalanine into tyrosine for use in the body. This defect leads to a build up of phenylalanine in the blood which causes neurological damage. Phenylalanine is found in most dietary protein. For this reason phenylketonurics have to maintain a low protein diet. They supplement their diet with a medical formula that provides protein without phenylalanine, as well as other nutrients. This way people with PKU can feel full and receive all the nutrients they need, despite a limited diet.

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2 thoughts on “PKU

  1. Hi am 28 years old I live in Sunderland north east of england I ment my husband 5 year And explained about Pku as I have this and we r planing a family this year And if any one got any repcits please pass them forward as am struggling with what I can eat many thanks Lorna Graham

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