Figure 3.

Glycosphingolipids pathways and location of intervention of substrate reduction therapy and chaperone therapy preparations. Dashed arrow indicates the mutated enzyme in FD. GM 1, 2, and 3 are gangliosides. Arrows indicate specific enzymatic reactions. Stop signs indicate point of intervention for each iminosugar. Red arrow indicates accumulation. *Migalastat inhibits αGAL A in high doses but aids proper folding of unstable αGAL A, resulting from certain amenable mutations, thus increasing enzymatic activity. FD, Fabry disease