Figure 4.

Hypothesized method of altered lipid homeostasis in Fabry disease. 1. Gb3 and cholesterol are taken up through the LDL receptor.102, 103, 104 2. Gb3 is mistargeted to lysosomes instead of Golgi and other membranes.105, 106, 107 3. Gb3 causes inhibition of ApoA1 mediated efflux of cholesterol.108 4. Storage of GSLs causes upregulation of the LDL receptor thus increasing intracellular uptake of cholesterol and additional GSLs.105, 106, 107 5. Increased uptake, mistargeting, and decreased efflux results in lysosomal accumulation of lipids. GSLs, glycosphingolipids; LDL, low‐density lipoprotein