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Lucerastat (Synonyms: NB-DGJ; N-(n-Butyl)deoxygalactonojirimycin)

Cat. No.: HY-106392
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Lucerastat, the galactose form of Miglustat, is an orally-available inhibitor of glucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study.

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

Lucerastat Chemical Structure

Lucerastat Chemical Structure

CAS No. : 141206-42-0

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Description

Lucerastat, the galactose form of Miglustat, is an orally-available inhibitor of glucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study[1][2].

In Vitro

Fabry patient-derived fibroblasts with the genotypes R301G (residual -GalA activity; 20%) R220X (<3%) and W162X (<1%).

Cell Viability Assay[2].

Cell Line: Fabry patient-derived fibroblasts with the genotypes R301G (residual -GalA activity; 20%) R220X (<3%) and W162X (<1%).
Concentration:
Incubation Time: 9 days.
Result: Dose-dependently inhibited GCS, reducing glucosylceramide and increasing sphingomyelin.
In Vivo

Lucerastat (1200 mg/kg/day food admix), a GCS inhibitor, reduces Gb3 in the absence of residual -GalA activity[2].

Animal Model: Fabry mice (Gla-/0 and Gla-/-, n = 5 or 6 for each gender)[2].
Dosage: 1200 mg/kg/day food admix.
Administration: Food admix for 20 weeks.
Result: Reduced lipid storage in two major organs affected by FD: mean Gb3 in the kidneys (-33%, p<0.01). and α-galactose- terminated glycosphingolipids in the dorsal root ganglia (-48%, p<0.05). In the liver of the Fabry mice, mean glucosylceramide (GlcCer (24:0)) was reduced (-59%, p<0.001) in addition to Gb3 (24:1) (-37%, p<0.05) demonstrating substrate reduction through GCS inhibition.
Molecular Weight

219.28

Formula

C₁₀H₂₁NO₄

CAS No.

141206-42-0

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

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Keywords:

LucerastatNB-DGJN-(n-Butyl)deoxygalactonojirimycinOthersFabrydiseaseglucosylceramidesynthaseInhibitorinhibitorinhibit

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