Question

5) Individuals with phenyl ketonuria are sensitive to phenyl alanine in their diet. Investigate this condition and why individuals with this condition are sensitive to aspartame, a sugar substitute

Answer 1

Phenylalanine is an essential amino acid. People suffering from Phenylketonuria (or PKU) are called phenylketonurics.People with PKU have a deficiency of an enzyme which is necessary for the proper metabolism of an amino acid called Phenylalanine.

The individuals with this condition are sensitive to aspartame because phenylalanine is found in aspartame, or simply we can say aspartame breaks down to various components in our body , one of which is phenylalanine.

If those of us that have PKU do not follow a strict low protein diet, phenylalanine is not adequately digested in our bodies and it builds up in our blood in the form of an acid called phenylpyruvic acid. High levels of phenylpyruvic acid damage the brain and can cause an array of side effects such as mental retardation, the loss of pigmentation in the skin/hair/eyes (people with untreated PKU often have light skin, blond hair and blue eyes), a musty odor, muscle aches and pains and in extreme cases, even seizures.