Phenylketonuria

Phenylketonuria (PKU) is a genetic disorder that prevents the normal use of protein food. There is an amino acid called phenylalanine found in many proteins. For a normal human, the extra (unneeded) phenylalanine accumulated through food is excreted or broken down. It is normally converted into tyrosine, another amino acid, by a specific organic catalyst (enzyme) called phenylalanine hydroxylase (PAH). Bodies of people born with PKU are unable to do so because their PAH enzyme …

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