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Sapropterin dihydrochloride Tablets is the prescription medication that helps many patients lower Phe levels....
MECHANISM OF ACTION
Inpheno is a synthetic form of BH4, the cofactor for the enzyme phenylalanine hydroxylase (PAH). PAH hydroxylates Phe through an oxidative reaction to form tyrosine. In patients with PKU, PAH activity
PhenylKetonUria (fen-ul-ke-toe-NU-re-uh) is an inborn error of metabolism that causes an amino acid known as phenylalanine (fen-ul-AL-uh-neen), or “Phe,” to build up in the blood because the phenylalanine hydroxylase (PAH) enzyme is either missing or not working properly.
Inpheno is the pharmaceutical form of BH4, the helper (or cofactor) of the PAH enzyme, which helps the PAH enzyme break down Phe. As a medication, Inpheno adds BH4 to the body, which helps lower Phe levels. Inpheno is available in tablet or powder dosage form, so you can choose the option that works best for you or your child.
Dosage : 100 mg/10 mg/customized dosage form
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Last Updated Nov 2014