Fig. 4

Vacuolar annexinopathy in FTLD-U due to ANXA11 p.P75S. a H&E stained sections revealed severe striatal degeneration with prominent vacuolization of the putamen (upper row, scale bars = 200, 50 and 100 µm, respectively), but with preservation of the substantia nigra (scale bar = 200 µm). GFAP stain highlights reactive astrocytosis. Ubiquitin and p62 immunohistochemistry revealed abundant neuritic inclusions (middle row). Annexin A11 aggregates affected many regions of the brain, including a high burden of dystrophic neurites in striatum (middle row), globular NCIs in the pons, dystrophic neurites in neocortex, and both dystrophic neurites and NCIs in the hippocampus (lower row, scale bar = 50 µm). b Double immunofluorescence showed striatal annexin A11 aggregates to be strongly ubiquitin positive (upper row) compared to neocortical regions (parietal cortex, lower row; scale bar = 50 µm)