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. 2017 Nov 25;18(12):2528. doi: 10.3390/ijms18122528

Figure 3.

Figure 3

Substrate reduction theory: Glucosylceramide synthase inhibitors are therapeutic targets for lipid storing diseases. Glucosylceramide synthase inhibitors (EtDO-P4, C10 and Eliglustat tartrate) halt the synthesis of glucosylceramide, thereby preventing the downstream synthesis and accumulation of different sphingolipids involved in diseases such as Gaucher, Fabry’s, Tay-Sachs and GM1-Gangliosidosis.