how are individuals with pku affected by phenylalanine? what effect would High l
ID: 3511084 • Letter: H
Question
how are individuals with pku affected by phenylalanine? what effect would High levels of phenylalanine have on a normal person who does not have the genetic condition of pku? how are individuals with pku affected by phenylalanine? what effect would High levels of phenylalanine have on a normal person who does not have the genetic condition of pku? how are individuals with pku affected by phenylalanine? what effect would High levels of phenylalanine have on a normal person who does not have the genetic condition of pku?Explanation / Answer
Answer:
Phenylketonuria(PKU)
Phenylalanine is an essential amino acid that gets converted into tyrosine with the help of an enzyme called phenylalanine hydroxylase. The deficiency of this enzyme causes a condition called phenylketonuria. In such individuals, either the enzyme as such is absent or the enzyme is defective.
Normal serum level is 1 mg/dl but in PKU it is elevated to more than 20 mg/dl.
It is a recessive condition. Phenyl lactic acid in sweat causes a mousy odor. Hypopigmentation is also noticed.
It is characterized by high levels of phenylalanine in the blood and hence they instead of becoming tyrosine, get converted into phenyl ketones(pyruvate), phenyl lactate and phenylacetate and are excreted in urine.
The main part of treating a patient with PKU is to control its intake with diet. Special diet with low phenylalanine like food based on cassava is adviced. During the first decade of life, special diet is mandatory although afterwards normal diet can be taken but cautiously. If the affected individuals are exposed to protein-rich diets with high phenylalanine content or even to artificial sweeteners such as aspartame, it can lead to serious health issues such as brain damage, mental deficiency and heightened neurological symptoms of seizures and convulsions, hyperactivity, agitation, psychologic and behavioral problems and intellectual disability.
For a person without a genetic condition of PKU, phenylalanine(in optimum quantities) do not get accumulated in blood because they have a normally functioning phenylalanine hydroxylase enzyme which converts phenylalanine into tyrosine which is used to make products such as melanin, catecholamines, and thyroxine. Intake of phenylalanine also elevates mood and increased energy levels in people. But excessive intake of high toxic doses can cause negative effects such as anxiety, hyperactivity, drug interactions with certain drugs(MAO) elevating blood pressure and nerve damage in normal healthy individuals without PKU genetics.
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