Acid ceramidase ((N-acylsphingosine deacylase, EC 3.5. 1.23) is the lysosomal enzyme
catalyzing the hydrolysis of ceramide to sphingosine and free fatty acid. Its inherited
deficiency causes ceramide accumulation in Farber's disease. The enzyme was purified to
apparent homogeneity from human urine by sequential chromatography on octyl-
Sepharose, concanavalin A-Sepharose, blue-Sepharose, and DEAE-cellulose. The final
preparation, which was enriched~ 4450-fold over the starting material, resulted in a …