产品描述:
Ibiglustat is an inhibitor of glucosylceramide (GlcCer) synthase.{57512} 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.{57513}