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

Art. -Nr.: HY-A0124: Data Sheet Handling Instructions
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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.

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Sapropterin Chemische Struktur

CAS. Nr. : 62989-33-7

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Other In-stock Forms of Sapropterin:

Sapropterin dihydrochloride

Other Forms of Sapropterin:

Tetrahydrobiopterin In-stock
Sapropterin dihydrochloride In-stock
Sapropterin-d₃ Angebot einholen
(6R)-Tetrahydro-L-biopterin sulfate Angebot einholen

2 Publications Citing Use of MCE Sapropterin

Spezialitätsempfehlungen

•Related Small Molecules:

•Related Proteins:

Alle Endogenous Metabolite Isoform-spezifische Produkte anzeigen:

Alle Isoformen anzeigen

Human Endogenous Metabolite Microbial Metabolite

Biologische Aktivität
Reinheit & Dokumentation
Verweise
Kundenbewertung

Beschreibung

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.

Molekulargewicht

241.25

Formel

C₉H₁₅N₅O₃

CAS. Nr.

62989-33-7

SMILES

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

Versand

Room temperature in continental US; may vary elsewhere.

Speicherung

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

Reinheit & Dokumentation

Handling Instructions (2659 KB)

Verweise

[1]. Levy H L, et al. Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin, 6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study[J]. The Lancet, 2007, 370(9586): 504-510. [Content Brief]

[2]. Schmitz K, et al. Sapropterin (BH4) Aggravates Autoimmune Encephalomyelitis in Mice. Neurotherapeutics. 2021;18(3):1862-1879. [Content Brief]

Sapropterin Related Classifications

Metabolic Disease Inflammation/Immunology Infection

Molaritätsrechner
Verdünnungsrechner

Die Formel zur Berechnung von Molaritäten

Mass (g) = Concentration (mol/L) × Volume (L) × Molecular Weight (g/mol)

Mass		Concentration		Volume		Molecular Weight *
	=		×		×

The dilution calculator equation

Konzentration _{(Stammlösung)} × Volumen _{(Stammlösung)} = Konzentration _{(Ziellösung)} × Volumen _{(Ziellösung)}

Diese Gleichung wird häufig abgekürzt als: C₁V₁ = C₂V₂

Concentration _(start)	×	Volume _(start)	=	Concentration _(final)	×	Volume _(final)
	×		=		×
C1		V1		C2		V2

Help & FAQs

Do most proteins show cross-species activity?
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.

Keywords:

Sapropterin62989-33-7(6R)-BH4 (6R)-Tetrahydro-L-biopterinEndogenous MetabolitePAHphenylalaninePKUSJL/J miceEAE miceT-cellautoimmune encephalomyelitisInhibitorinhibitorinhibit

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