The subsequent overaccumulation of storage material may also physically damage the lysosomal membranes, causing dispersal of storage contents into the cytosol or the extracellular space and subsequent inflammasome activation and neuronal cell death, as it was shown recently in a genetic model for acid sphingomyelinase deficiency and in a demyelination model (Cantuti-Castelvetri et al., 2018; Gabandé Rodríguez et al., 2018). Here, SMPD1 is linked to hyperinsulinemic hypoglycemia, familial, 4.