What is the function of phenylalanine?

Function: The essential amino acid L-phenylalanine (Phe) is needed for the synthesis of proteins, catecholamines, and melanin; it is also an important precursor of the amino acid L-tyrosine (Tyr).

What is phenylalanine deficiency?

Phenylalanine hydroxylase deficiency (PAH deficiency), also called phenylketonuria (PKU), is an inherited disease in which the body cannot properly process the amino acid phenylalanine due to a deficient enzyme called phenylalanine hydroxylase.

What is meant by phenylalanine?

Medical Definition of phenylalanine : an essential amino acid C9H11NO2 that is obtained in its levorotatory L-form by the hydrolysis of proteins (as lactalbumin), that is essential in human nutrition, and that is converted in the normal body to tyrosine —abbreviation Phe — see phenylketonuria, phenylpyruvic acid.

What part of the body does PKU affect?

A. Phenylketonuria (PKU) is a treatable disorder that affects the way the body processes protein. Children with PKU cannot use a part of the protein called phenylalanine. If left untreated, phenylalanine builds up in the bloodstream and causes brain damage.

How does PKU affect the brain?

PKU affects the brain. When neurotransmitters are not made in the right amounts, the brain cannot function properly. High blood Phe levels can cause disruptions in neurotransmitters like serotonin and dopamine, which are important for mood, learning, memory, and motivation.

Why does phenylalanine cause mental retardation?

Mutations in the PAH gene can cause phenylketonuria (PKU), a disorder that can change cells in the brain. The faulty protein allows dangerously high levels of phenylalanine to accumulate in the brain, poisoning the cells. If a person with PKU consumes too much phenylalanine, the build-up can cause mental retardation.

What is another word for phenylalanine?

In this page you can discover 17 synonyms, antonyms, idiomatic expressions, and related words for phenylalanine, like: ornithine, amino-acid, methionine, tryptophan, , tyramine, arginine, lysine, pyruvate, carnitine and rhamnose.

Why is phenylalanine neurotoxic?

The high plasma phenylalanine concentrations increase phenylalanine entry into brain and restrict the entry of other large neutral amino acids. In the literature, emphasis has been on high brain phenylalanine as the pathological substrate that causes mental retardation.