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4 Aromatic Amino Acids in the Brain - Wurtman Lab
4 Aromatic Amino Acids in the Brain - Wurtman Lab

... Abstract: This chapter describes the aromatic L‐amino acids tryptophan and tyrosine and the effects on tyrosine metabolism of phenylalanine. Tryptophan and phenylalanine are essential amino acids and must ultimately be derived from dietary proteins; tyrosine is obtained both from dietary proteins an ...
Dopamine File
Dopamine File

... with Parkinson’s disease, which is characterised by tremor and uncontrolled movements. It is treated with L-dopa (laevodopa), which is a precursor for dopamine. In contrast, high levels of dopamine are linked to schizophrenia, increased arousal, reduced inhibition and increased creativity. • Q Sugge ...
journal - Breining Institute
journal - Breining Institute

... reaction is perfectly normal and helpful for survival. At other times, the symptoms caused by a surge in adrenaline can be distressing and frightening. A person having a panic attack often is overwhelmed by a sense of impending disaster or death. Symptoms which may accompany a panic attack include s ...
An Animal Model of Early-treated PKU
An Animal Model of Early-treated PKU

Human Heredity
Human Heredity

... Huntington’s Disease ...
1

Phenylketonuria



Phenylketonuria (PKU) (phenyl + ketone + -uria; /ˌfinəlˌkitɵnˈjʊəriə/) is an inborn error of metabolism involving impaired metabolism of phenylalanine, one of the amino acids. Phenylketonuria is caused by absent or virtually absent phenylalanine hydroxylase (PAH) enzyme activity. The condition is also known as ""phenylalanine hydroxylase deficiency.""Protein-rich foods or the sweetener aspartame can act as poisons for people with phenylketonuria. The role of PAH is to break down excess phenylalanine from food. Phenylalanine is a necessary part of the human diet and is naturally present in all kinds of dietary protein. It is also used to make aspartame, known by the trade name Nutrasweet, which is used to sweeten low-calorie and sugar free soft drinks, yogurts, and desserts. In people without PKU, the PAH enzyme breaks down any excess phenylalanine from these sources beyond what is needed by the body. However, if there is not enough of the PAH enzyme or its cofactor, then phenylalanine can build up in the blood and brain to toxic levels, affecting brain development and function. PKU is rare, but important to identify, because if caught early it is very treatable. It is not contagious, and it is lifelong, but with early diagnosis and consistent treatment, the damaging effects can be minimal or non-existent.Untreated PKU can lead to intellectual disability, seizures, and other serious medical problems. The best proven treatment for classical PKU patients is a strict phenylalanine-restricted diet supplemented by a medical formula containing amino acids and other nutrients. In the United States, the current recommendation is that the PKU diet should be maintained for life. Patients who are diagnosed early and maintain a strict diet can have a normal life span with normal mental development.PKU is an inherited disease. When an infant is diagnosed with PKU, it is never the result of any action of the parents or any environmental factor. Rather, for a child to inherit PKU, both of his or her parents must have at least one mutated allele of the PAH gene. Most parents who are carriers of PKU genes are not aware that they have this mutation because being a carrier causes no medical problems. To be affected by PKU, a child must inherit two mutated alleles, one from each parent.
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