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.
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.
Essential amino acids are those the body cannot produce. There are 20 basic amino acids. There are eight essential amino acids unless the individual is a phenylketonuric. There are nine essential amino acids for phenylketonurics as they cannot convert phenylalanine to tyrosine. Essential amino acids have to be taken into the body by eating. This answer is for informational purposes only. All individuals should ask advice of their physician.
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The Acceptable Daily Intake (ADI) for Aspartame is 40 mg per kg of body weight. For example, a 70 kg person can safely consume 2800 mg of Aspartame per day. This is equivalent to roughly 14 cans of diet soda. It is important to monitor total intake from all sources to ensure you are within safe limits.
Essential amino acids are those that the body cannot produce and must be obtained from the diet. On the other hand, non-essential amino acids can be synthesized by the body. Both types of amino acids are crucial for synthesizing proteins and supporting various biological functions in the body.
In humans, disorders of phenylalanine or tyrosine metabolism include phenylketonuria (PKU) and alkaptonuria. PKU is caused by a deficiency in the enzyme phenylalanine hydroxylase, leading to a buildup of phenylalanine in the body. Alkaptonuria is due to a deficiency in homogentisate 1,2-dioxygenase, resulting in the accumulation of homogentisic acid. Both disorders can have serious health consequences if not managed properly.
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