A GlcCer synthase inhibitor
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Ibiglustat

Item No. 32878

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
N-[1-[2-(4-fluorophenyl)-4-thiazolyl]-1-methylethyl]-carbamic acid, (3S)-1-azabicyclo[2.2.2]oct-3-yl ester
CAS Number
1401090-53-6
Synonyms
  • Genz-682452
  • GZ/SAR402671
  • Venglustat
Molecular Formula
C20H24FN3O2S
Formula Weight
389.5
Purity
≥98%
Formulation
A crystalline solid
Solubility(Learn about Variance in Solubility)
DMF: 30 mg/mlDMSO: 25 mg/mlEthanol: 30 mg/mlEthanol:PBS (pH 7.2) (1:7): 0.1 mg/ml
λmax
293 nm
SMILES
CC(C)(NC(O[C@@H]1CN2CCC1CC2)=O)C3=CSC(C4=CC=C(F)C=C4)=N3
InChi Code
InChI=1S/C20H24FN3O2S/c1-20(2,17-12-27-18(22-17)14-3-5-15(21)6-4-14)23-19(25)26-16-11-24-9-7-13(16)8-10-24/h3-6,12-13,16H,7-11H2,1-2H3,(H,23,25)/t16-/m1/s1
InChi Key
YFHRCLAKZBDRHN-MRXNPFEDSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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    25 mg

    $141

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    50 mg

    $268

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    100 mg

    $478

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    250 mg

    $773

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    Product Description

    Ibiglustat is an inhibitor of glucosylceramide (GlcCer) synthase.1 Dietary administration of ibiglustat (0.03% w/w) reduces globotriaosylceramide (Gb3) and lyso-Gb3 accumulation in a variety of tissues, including the kidney, heart, and brain, in the Gla-/- mouse model of Fabry disease. It reduces the latency to paw withdrawal in the hot plate test in the same model, indicating reduced peripheral neuropathy. Ibiglustat (0.03% w/w in the diet) reduces gliosis, delays the onset of ataxia, and increases lifespan in the transgenic 4L;C* mouse model of neuronopathic Gaucher disease.2

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Ashe, K.M., Budman, E., Bangari, D.S., et al. Efficacy of enzyme and substrate reduction therapy with a novel antagonist of glucosylceramide synthase for Fabry disease. Mol. Med. 21(1), 389-399 (2015).

    2. Marshall, J., Sun, Y., Bangari, D.S., et al. CNS-accessible inhibitor of glucosylceramide synthase for substrate reduction therapy of neuronopathic Gaucher disease. Mol. Ther. 24(6), 1019-1029 (2016).