Biological Activity
Ibiglustat (Venglustat) is an inhibitor of glucosylceramide synthase extracted from patent WO 2015089067 A1, compound example 64.
In Vitro
No in vitro data are provided in the current product source. Contact us for further information.
In Vivo
Ibiglustat is in phase II clinical trials for the treatment of Fabry's disease. Fabry disease, an X-linked disorder of glycosphingolipids that is caused by mutations of the GLA gene that codes for α-galactosidase A, leads to dysfunction of many cell types and includes a systemic vasculopathy[2]. Substrate reduction therapy is an approach that reduces the synthesis of lipids reaching the lysosome through inhibition of glucosylceramide synthase (GCS)[1].
References & source
Published experimental findings depend on the model and assay conditions. They do not guarantee equivalent performance in every application. For laboratory research use only.
