UGCG and Gaucher disease: Controlling in vivo GlcCer synthesis can modulate substrate level in glycosphingolipids storage disease mice and change the disease outcome, e.g., pharmacological inhibition of GCS as in substrate reduction therapy (SRT) in the mouse models of Gaucher disease [36], Sandhoff disease [37], Fabry disease [38] and GM1 gangliosidosis [39].