Figure 3.

GM3, GD3, and GM1 ganglioside accumulation in cerebral brain tissue of the Mcoln1^−/− mouse by immunohistochemistry. (A, B) Hemi-coronal cerebral brain sections immunoperoxidase labeled for GM3 and Nissl counterstained showed no accumulation in the CA3 region of the hippocampus of a wild type (Wt) mouse (A), but significant accumulation in a Mcoln1^−/− mouse (B). (C, D) Similarly, high magnification images of cortical pyramidal neurons showed no GM3 in a Wt mouse (C), but perinuclear accumulation of GM3 in neurons of the Mcoln1^−/− mouse (D). (E–I) Immunoperoxidase labeling for GD3 in the amygdala showed no accumulation in a Wt mouse (E) but significant accumulation in the Mcoln1^−/− mouse (F). GD3 accumulation was also absent in the CA1/CA2 region of the hippocampus of a Wt mouse (G) but is conspicuous throughout this region of a Mcoln1^−/− mouse (H). Cortical pyramidal neuron of the Mcoln1^−/− mouse (I) showed large amounts of GD3 accumulation throughout the perikaryon as well as in the axon hillock, apical dendrite, and other neuritic processes. (J–L) GM1 ganglioside appeared redistributed in an intracellular vesicular pattern in cortical pyramidal neurons of the Mcoln1^−/− mouse (K, M) compared to a Wt mouse (J, L) by immunoperoxidase labeling. Scale bars: A and G = 50 μm and also pertain to B and H, respectively; C and L = 5 μm and pertain to D and M, respectively; E and J = 10 μm and pertain to F and K, respectively; I = 5 μm. Nucleus, N; axon hillock, arrowhead; perikaryon, PK; apical dendrite, arrow.