Figure 2
GM 2 ganglioside storage in the Thy1-HEXB;HexB −/− brain. (A) Wild-type mice did not display any GM2 storage as detected by immunohistochemistry (black staining) employing a monoclonal antibody. (B) The expression of human HEXB in the neurons of HexB−/− mice driven by the Thy1 promoter resulted amelioration of GM2 storage, as seen by the lack of any significant GM2 immunoreactivity in 4-month-old Thy1-HEXB;HexB−/− mouse brain compared to (C) HexB−/− littermates. (D) Glial fibrillary acidic protein (GFAP)+ astrocytes (E) did not contain, as detected by the lack of double immunofluorescence, the presence of (F) GM2 ganglioside. However, (G) major histocompatibility complex class II (MHC-II)+ microglia/macrophages contained, as detected (H) by double immunofluorescence, the presence of (I) GM2 ganglioside. Bar = 100 μM. HEXB = β-hexosaminidase β/β homodimer.