Figure 5

EM analysis reveals degenerative changes in R1098Q PCs and the molecular layer. (a) The PC’s of R1098Q 1.5 yo mice exhibit massively dilated organelle structures that appear to arise from Golgi and ER, along with dispersal of Nissl substance. Many multivesicular bodies (mvb) and multilamellar bodies (mlb) are present, presumably derived from autophagocytic vacuoles. Mitochondia (arrows) also frequently show internal vesiculation.(b) Within the cerebellar molecular layer of the heterozygous animals, dendrites are swollen and post synaptic densities (PSD) are attenuated and sparse. (c) The PSD’s identified in eight separate EM images of WT mouse and eight images from a heterozygous R1098Q mouse were counted. There is an approximately four-fold reduction in PSD’s in the heterozygous 1.5 yo mouse. The variation in the replicates comparing these two mice were evaluated by paired single tail T-test.