Fig. 2

Viral expression of ubiquilin-2 at 6 months. Representative schema of sagittal section shows the overall distribution of AAV-UBQLN2 expression in mouse brain after ICV injection (SBT model). Photos show ubiquilin-2 immunostaining in representative sections animals injected with AAV expressing either EGFP control or WT vs P497S, P497H, or P506T mutant ubiquilin-2. WT ubiqulin-2 expression is homogeneous throughout the neuronal perykaria, including processes. Mutant ubiquilin-2 shows altered subcellular expression, often concentrating in the nucleus, but also resulting cytoplasmic inclusions. Ubiquilin-2-positive “aggregates” are seen also in adjacent neuropil. Arrows point out alteration in purkinje cell dendritic arbors for mutant vs WT ubiquilin-2. (Scale same for all photomicrographs, bar = 50 μm)