产品描述:
Conduritol B epoxide (CBE) is an irreversible inhibitor of glucocerebrosidase, also known as acid β-glucosidase, glucosylceramidase, GBA, or GBA1 (IC50 = 9 μM).{25307,25308} Inhibition of this lysosomal glucosidase results in the accumulation of glucocerebroside without affecting cell viability, lysosomal enzyme release, or the activity of intracellular enzymes.{25309} It also does not inhibit non-lysosomal glucosylceramidase or cytosolic β-glucosidase.{25306} As glucocerebrosidase deficiency results in Gaucher disease, a common lysosomal storage disorder that can involve defects in blood, bone, neurological, and liver development, CBE is used in in vitro and animal models of the disease.{25308,25306}