Figure 3

Neuropathology of SBT FUS _Δ14 mice is similar to human FUS proteinopathies (A-P). Adjacent sections from the cerebral cortex of eGFP (A-D), FUS_WT (E-H), FUS_R521C (I-L), and FUS_Δ14 (M-P) mice stained with protein NCI markers found in aFTLD-U, NIFID, BIBD and hematoxylin-eosin (H&E). (M) Only FUS_Δ14 brains contain ubiquitinated NCIs. (N) Hematoxylin-eosin (H&E) staining of FUS_Δ14 cerebral cortex shows cytoplasmic basophilic inclusions (arrows). (O) NCIs are infrequently positive for α-internexin, similar to the pathological NCIs found in NIFID cases. (P) Some NCI in the cerebral cortex of FUS_Δ14 mice contain the stress granule marker protein PABP-1. Scale bar: 100 μm. Slides are orientated to a common blood vessel (arrow head) to serve as a landmark (M-P).