Sapropterin dihydrochloride

- Overview
  - Sapropterin dihydrochloride [formerly known as Tetrahydrobiopterin (BH4, THB, trade name: Kuvan) or sapropterin] is a synthetic preparation of the dihydrochloride salt of naturally occurring tetrahydrobiopterin (BH4) that is approved for the treatment of BH4 responsive PKU. It is a phenylalanine hydroxylase activator. Sapropterin is a naturally occurring essential cofactor of the three aromatic amino acid hydroxylase enzymes, used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine, norepinephrine (noradrenaline), epinephrine (adrenaline), and is a cofactor for the production of nitric oxide (NO) by the nitric oxide synthases. Chemically, its structure is that of a reduced pteridine derivative.
    
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- Properties
  - Categories
    
    Disease Research Fields
    
    CAS Number
    
    69056-38-8
    
    Molecular Formula
    
    C9H17CL2N5O3
    
    Molecular Weight
    
    314.17
    
    Purity
    
    ≥98%
    
    Storage
    
    Powder: store in -20°C freezer for up to three years, store in 4°C freezer for up to two years
    In solvent: store in -80°C freezer for up to six months, store in -20°C freezer for up to two months
    
    Shipping
    
    Room temperature (This product is stable at ambient temperature for a few days during ordinary shipping and time spent in Customs)
    
    SMILES
    
    O=C1N=C(N)NC2=C1N[C@@H]([C@@H](O)[C@@H](O)C)CN2.[H]Cl.[H]Cl
    
    * For research use only

Sapropterin dihydrochloride

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