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Tetrahydrobiopterin (BH4) or also known as sapropterin is a synthetic analog of the natural 6R-BH4 which is responsible for many essential processes in the body. It has a big role in the functioning of the heart, brain, digestive and reproductive systems. It is used to treat patients with hyperphenylalaninemia suffering from phenylketonuria or from a deficiency of BH4. Hyperphenylalaninemia is a disease characterized by an increased level of phenylalanine in the blood and the reason for this are autosomal recessive mutations of the genes that encode the enzyme phenylalanine hydroxylase (in the case of phenylketonuria) or enzymes involved in biosynthesis or regeneration of the 6R-BH4 (in the case of BH4 deficiency). BH4 deficiency is a group of diseases associated with mutations or gene deletions that encode one of the five enzymes involved in the biosynthesis or recycling of BH4. In both cases, phenylalanine cannot be effectively converted to the amino acid tyrosine, resulting in increased blood levels of phenylalanine. Along with the BH4 treatment, it is required an active control of phenylalanine and total protein received from food to ensure adequate control of phenylalanine level in the blood and nutrient balance. You can buy Tetrahydrobiopterin (BH4) online on our website and we guarantee fast shipping to USA or UK and cheap prices.