Development of an acid ceramidase activity-based probeElectronic supplementary information (ESI) available: Experimental and analytical details on the synthesis of the compounds, and experimental biochemical procedures. See DOI: 10.1039/c5cc00356c

Acid ceramidase is responsible for the ultimate step in the catabolism of (glyco)sphingolipids by hydrolysis of ceramide into sphingosine and free fatty acid. Deficiency in acid ceramidase is the molecular basis of Farber disease. Here we report the synthesis and characterization of an activity-based acid ceramidase probe. Acid ceramidase is responsible for the ultimate step in the catabolism of (glyco)sphingolipids by hydrolysis of ceramide into sphingosine and free fatty acid.