IL1B and Sandhoff disease: Expression levels of the astrocyte marker, Gfap, and the myeloid-lineage cell marker, Adgre1 (F4/80), as well as the inflammatory chemokines Ccl3, Ccl5, Gpnmb, Il-1β, and Tnfα were up-regulated in cerebral hemispheres obtained from the most severe stage of mouse models of nGD [21], Krabbe’s disease [44], Niemann-Pick type C1 disease (NPC1) [5] and Sandhoff disease [45], indicative of neuroinflammation (Table 2).