Sapropterin Dihydrochloride (BH4) | CAS 69056-38-8

Product Name: Sapropterin Dihydrochloride (BH4)
CAS No.: 69056-38-8
Purity: ≥99.0%

Description

Tetrahydrobiopterin (BH4, THB), also known as sapropterin (INN), is a cofactor for three aromatic amino acid hydroxylase enzymes. It is involved in the breakdown of the amino acid phenylalanine and in the biosynthesis of several important neurotransmitters and hormones, including:

• Serotonin (5-hydroxytryptamine, 5-HT)

• Melatonin

• Dopamine

• Norepinephrine (noradrenaline)

• Epinephrine (adrenaline)

BH4 is also a cofactor for nitric oxide (NO) production via nitric oxide synthase.

What is Sapropterin?

Sapropterin is used together with a restricted diet to control blood phenylalanine levels in adults and children from 1 month of age with phenylketonuria (PKU) – a congenital disorder in which phenylalanine accumulates in the blood, potentially causing reduced intellectual ability and difficulties with concentration, memory, and information processing.

Sapropterin is suitable only for certain PKU patients. The only way to determine if sapropterin will help a specific patient is to administer the drug for a period of time and observe whether blood phenylalanine levels decrease.

Mechanism of Action

Sapropterin belongs to a class of medications called cofactors. It helps the body break down phenylalanine, preventing its accumulation in the blood.

Brand Names and Formulations

Tetrahydrobiopterin is available as oral tablets and powder in the form of sapropterin dihydrochloride (BH4·2HCl).

• Approved in: United States (December 2007 for tablets, December 2013 for powder), European Union (December 2008), Canada (April 2010), Japan (July 2008)

• Brand names: Kuvan®, Biopten®

API Supply

High-purity sapropterin API (≥99%) is available for research and pharmaceutical development purposes.