Conduritol-B-Epoxide (CBE)-Induced Gaucher Disease
Gaucher disease (GD) is the most common lysosomal storage disorder. The main pathological hallmark is the intracellular accumulation of glucosylceramide and glucosphingosine as a result of reduced glucocerebrosidase (GCase) enzyme activity due to mutations in the β-glucocerebrosidase gene (GBA).
Conduritol-beta-epoxide (CBE) is a specific inhibitor of GCase activity and can thus be used to induce Gaucher disease in vivo.
C57Bl/6 mice were intraperitoneally injected with 100 mg/kg CBE on 9 consecutive days and brains analyzed for neuroinflammation.
Figure 1. Cortical astrocytosis and activated microglia of CBE-treated mice. GFAP (A) and CD11b (B) immunoreactive (IR) area in percent. n = 9 per group; unpaired t-test; Mean + SEM. ***p<0.001. Representative images of cortical tissue of vehicle-treated or CBE-treated mice. Note the occurrence of extremely enlarged microglia (arrowhead) and increased GFAP in CBE-treated mice.