Fig. 3
From: Clinical and neuropathological features of ALS/FTD with TIA1 mutations
TDP-43 immunoreactive pathology in TIA1 mutation carriers. Numerous predominantly granular TDP-43 immunoreactive (TDP-ir) neuronal cytoplasmic inclusions (NCI, arrows) were present in the prefrontal cortex (a) and primary motor cortex (b). Hippocampal dentate granule cells (c) and dopaminergic neurons in the substantia nigra (d) were consistently affected in all cases. Lower motor neurons (LMN) of the medulla and spinal cord (e – i) contained NCI that were granular (e), filamentous (f) or round and compact (g and h). Single LMN containing combinations of NCI types were not uncommon (i, arrow points to filamentous inclusions in close proximity to a compact, round NCI). Phosphorylation-independent TDP-43 immunohistochemistry. Scale bar: a and i, 25 μm; b, c and e, 18 μm; d and g, 36 μm; f and h, 9 μm