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Lysosomal SL catabolism and enzyme deficiencies causing storage diseases. A schematic of the various SL metabolic pathways is presented, indicating the enzymes whose deficiency leads to several diseases. Note that each enzyme is assisted by one or more Saps: GM2A assists both β-gal and β-hexosaminidase. SapB assists sialidase, α-GAL, GALC, and β-gal. SapC assists GALC, β-gal, GCase, acid ceramidase, and GALC. Acid ceramidase is also assisted by SapD
