The lysosomal acid ceramidase (AC, ASAH1, Figure 2) is an Ntn-hydrolase deacetylating ceramide to generate sphingosine and free fatty acid, so that AC deficiency leads to the lysosomal storage disorder Farber disease (FD; lipogranulomatosis) [64] and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME), where the latter is a rare condition with only around 200 cases reported [65]. Here, ASAH1 is linked to Erdheim-Chester disease.