The sphingolipid (SL) metabolic pathway generates structurally diverse lipids that have roles
as membrane constituents and as bioactive signaling molecules. The influence of the SL
metabolic pathway in biology is pervasive; it exists in all mammalian cells and has roles in
many cellular and physiological pathways. Human genetic diseases have long been
recognized to be caused by mutations in the pathway, but until recently these mutational
defects were only known to affect lysosomal SL degradation. Now, with a nearly complete …