Tetrahydrobiopterin is a vital nutrient that aids in the creation of essential neurochemicals. Its deficiency leads to cognitive problems and impaired motor function, as seen in phenylketonuria (PKU). Kuvan, a prescription drug, has been created for the treatment of PKU.
Tetrahydrobiopterin is a natural nutrient that is essential in the human body. It is important as a cofactor in several enzymes required for proper mental function. The nutrient is also known as THB, BH4, and sapropterin.
This key nutrient is synthesized from guanosine triphosphate (GTP), which is used as an energy source for protein synthesis. Tetrahydrobiopterin’s function is to aid in the creation of essential neurochemicals. Serotonin, adrenaline, and nitric oxide (NO) are synthesized by enzymes that cannot be created without the presence of tetrahydrobiopterin.
The lack of it, or limited production, leads to tetrahydrobiopterin deficiency. When a limited amount of the substance is present in the human body, the essential amino acid phenylalanine will begin to accumulate. This acid is obtained through food and is extremely common. In the body, phenylalanine is converted to phenylalanine hydroxylase (PAH), which is then converted to an enzyme by its interaction with tetrahydrobiopterin. Without enough tetrahydrobiopterin to interact, PAH supplies in the body will be maximized and phenylalanine will begin to accumulate. This condition usually presents at a very young age and leads to cognitive problems and impaired motor function.
There is also a genetic disorder known as phenylketonuria (PKU) that shows similar symptoms. PKU makes it difficult for the body to produce PAH, which in turn means a buildup of phenylalanine. Also, a limited amount of PAH means that there will be a limited amount of the enzyme tyrosine produced, which is created when tetrahydrobiopterin is combined with PAH. This enzyme is essential in the creation of dopamine and norepinephrine, two essential neurotransmitters. The condition was discovered in 1934 by Norwegian physician Ivar Asbjorn Folling, and is also known as Folling’s disease. In the developed world, most babies are tested for this condition between six and 14 days of age, using a simple blood test.
A prescription drug known as Kuvan has been created by the BioMarin pharmaceutical corporation for the treatment of PKU. In the United States, the Food and Drug Administration (FDA) approved this drug for sale on December 13, 2007. The proprietary process used to create this drug uses the salt form of tetrahydrobiopterin, and has been shown in clinical trials. It reduces the amount of phenylalanine levels in the blood. With the use of both drug treatments and a strict diet, PKU can usually be controlled and its symptoms controlled.
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