Phenylalanine

Phenylalanine

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Phenylalanine

Phenyklketonuria is an autonomic recessive disorder. It is caused by a shortage in the enzyme "Phenylalanine Hydroxylase". It is a genetic disorder which prevents the normal use of protein food, and is also present at birth as a single disorder, mainly caused by parents. (Antoniou and Katsambas, 2006,490-498)

Each parent of a child with PKU carries one defective gene for the disorder and one normal gene. When each parent produces sperm or eggs, only one of their two PAH (Phenylalanine Hydroxylase) genes goes into each cell. Half of these cells will contain mutated PAH. When the sperm and egg unite which both have a PAH gene, they produce a child with two mutated genes. Not every child of the couple has to be left the disease. Other children of the couple with one defective gene and one normal gene have a chance to be unaffected, but live as a carrier. The chances of this are twenty five percent. There is also a one in four chance that both will pass on the normal gene, and the baby will neither have the disease nor be a carrier. These chances are the same in each pregnancy. Boys and girls have the same chance of risk of inheriting this disorder. (Antoniou and Katsambas, 2006,490-498)

Without a good PAH enzyme, the person can gain high phenylalanine in the brain. In PKU one of the enzymes are unable to convert PAH into Tyrosine (another amino acid). The lack of tyrosine results to Phenylalanine poison, which causes retardation and epilepsy. If the disease is not looked at and treated on time, it will cause brain damage. Children that aren't treated become obvious in the first month. Phenlyketonuria is the result of a variety in the shortages to other enzymes that are closely related to phenylalanine hydroxlase. PKU appears in about 1 in 10,000 births in Caucasians and East Asians. The actions can be found to a small mutation in a single gene on chromosome 12.

An early discovery of PKU is very important because signs aren't obvious in a newborn baby. Symptoms of PKU usually develop within a few months after birth, when the phenylalanine has completed up in a babies system from taking the protein in formula or breast milk. (Antoniou and Katsambas, 2006,490-498)

Phenylalanine is a hidden danger to anyone consuming aspartame. Most consumers don't know that too much Phenylalanine is a neurotoxin and excites the neurons in the brain to the point of cellular death. ADD/ADHD, emotional and behavioral disorders can all be triggered by too much Phenylalanine in the daily diet. If you are one in ten thousand people who are PKU or carry the PKU gene, Phenylalanine can cause irreversible brain damage and death, especially when used in high quantities or during pregnancy. Phenylalanine is 50% of aspartame, and to the degree humans consume diet products, Phenylalanine levels are reaching a dangerous peak. (Antoniou and Katsambas, 2006,490-498)

It is important to learn about the ingredients within your foods, especially isolated ...