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  3. Sapropterin

Sapropterin  (Synonyms: (6R)-BH4; (6R)-Tetrahydro-L-biopterin)

Cat. No.: HY-A0124
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Sapropterin ((6R)-BH4; (6R)-Tetrahydro-L-biopterin) is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin can be used in study of phenylketonuria (PKU) phenylketonuria. Sapropterin can aggravate experimental autoimmune encephalomyelitis (EAE).

At equivalent molar concentrations, both the salt and free forms of a compound exhibit comparable biological activity. Nevertheless, the salt form (Sapropterin dihydrochloride) usually boasts enhanced water solubility and stability.

For research use only. We do not sell to patients.

Sapropterin

Sapropterin Chemical Structure

CAS No. : 62989-33-7

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Description

Sapropterin ((6R)-BH4; (6R)-Tetrahydro-L-biopterin) is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin can be used in study of phenylketonuria (PKU) phenylketonuria. Sapropterin can aggravate experimental autoimmune encephalomyelitis (EAE)[1][2].

IC50 & Target

Human Endogenous Metabolite

 

In Vitro

Sapropterin enhances residual phenylalanine hydroxylase activity to increase phenylalanine oxidation[1].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

In Vivo

Sapropterin (100 mg/kg/day; oral; daily; 22 days) treatment aggravates primary progressive and relapsing-remitting EAE in SJL/J mice, as evidenced by increased clinical EAE scores, elevated plasma long-chain ceramide levels, reduced linolenic acid levels, and increased spinal cord T-cell infiltration[2].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

Animal Model: SJL/J (female, 10-12-week-old, primary progressive and PLP-induced-relapsing-remitting EAE)[2]
Dosage: 100 mg/kg/day
Administration: oral; daily; 22 days
Result: Reached higher maximum clinical EAE scores and showed a significant increase in the area under the curve (AUC) of EAE scores.
Elevated plasma levels of long-chain ceramides (C14Cer, C16Cer, C18Cer, C20Cer, C18GluCer, C24:1GluCer).
Reduced plasma linolenic acid (FA18:3) levels and increased plasma levels of the endocannabinoid 1+2AG.
Showed no significant effect on peripheral T-cell proliferation or subpopulations in the spleen, but increased infiltration of CD4+ and CD8+ T-cells in the spinal cord.
Molecular Weight

241.25

Formula

C9H15N5O3

CAS No.
SMILES

O=C1C2=C(NC(N)=N1)NC[C@](N2)([H])[C@@H](O)[C@@H](O)C

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Room temperature in continental US; may vary elsewhere.

Storage

Please store the product under the recommended conditions in the Certificate of Analysis.

Purity & Documentation
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    Species cross-reactivity must be investigated individually for each product. Many human cytokines will produce a nice response in mouse cell lines, and many mouse proteins will show activity on human cells. Other proteins may have a lower specific activity when used in the opposite species.

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