N-Butyldeoxynojirimycin hydrochloride

CAS: 210110-90-0

Ref. 3D-MB04673

Produktinformation

• Name: N-Butyldeoxynojirimycin hydrochloride
• Synonyme: Miglustat hydrochloride
• Marke: Biosynth

Beschreibung

Competitive inhibitor of ceramide-glycosyltransferase used for substrate reduction therapy in lysosomal storage disorders. It inhibits glucosylceramide synthase, which catalyses the initial step in glycosphingolipid biosynthetic pathway. This compound delays the onset of symptoms in type 1 Gaucher disease, Sandhoff disease and Tay-Sachs disease. It also reduces brain abnormalities in mucolipidosis type IV.

Chemische Eigenschaften

• Molekulargewicht: 255.74 g/mol
• Formel: C₁₀H₂₁NO₄•HCl
• Reinheit: Min. 95%
• Farbe/Form: Powder