The term "phenylketonuric" is an individual whose body cannot process certain substances (phenylalanine) into the amino acid tyrosine. This can cause a toxic buildup of phenylketone (aka phenylpyruvate) in the body tissues.
The condition is a genetic defect that is ordinarily treated by dietary changes. It has gained prominence due to warnings for the artificial sweetener aspartame (Nutrasweet, Equal). Because the human body breaks down the sweetener into other substances, including phenylalanine, the consumption of the product could elevate the level of that chemical in phenylketonuric individuals.
Phenylketonuria (PKU) is an autosomal recessive genetic disorder characterized by a deficiency in the enzyme phenylalanine hydroxylase (PAH). This enzyme is necessary to metabolize the amino acid phenylalanine to the amino acid tyrosine. When PAH is deficient, phenylalanine accumulates and is converted into phenylpyruvate (also known as phenylketone), which is detected in the urine.
Definition is the same as meaning.
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