Interestingly, a different transport mechanism mediated by the lysosomal integral membrane protein 2 (LIMP2/SCARB2) has been identified for the lysosomal enzyme β-glucocerebrosidase, which is responsible for the hydrolysis of the simplest GSL at its glycosidic bond, i.e. glucosylceramide, in Glc and ceramide, a deficiency being the cause for the lysosomal storage disorder Gaucher’s disease [84]. Here, SCARB2 is linked to Gaucher disease.