Figure 5.
Attenuation of GM1-ganglioside storage in the brain caused by exogenously delivered β-gal. (A) Thin-layer chromatography of hindbrain, midbrain, and forebrain (HMF), brain stem (BS), and cerebellum (CB) of Glb1+/+, Glb1-/-, and treated Glb1-/- mice 3 months after BMT demonstrated amelioration of GM1-ganglioside storage in the brain stem and cerebellum of the treated mice. The attenuation of GM1-ganglioside accumulation was in agreement with the increment of β-gal activities in those areas (bottom row). GM1-ganglioside was used as a standard. (B) Confocal microscopy with anti-GM1 antibody confirmed less GM1 accumulation in the cerebellum, brain stem, and hippocampus of treated mice (Glb1-/- BMTβ-gal) than in Glb1-/- mice that underwent mock transplantation (Glb1-/- BMTGFP). The arrows indicate cells in which GM1-ganglioside accumulation was most arrested. Size bar corresponds to 50 μm. Image acquisition was performed as described for Figure 3.