Fig. 1

Microgliosis is observed throughout the motor cortex of ALS patients with TDP-43 pathology. Representative images of layer 5 of the motor cortex isolated from postmortem normal controls and ALS patients. Representative image of cortical neuron showing neuronal cytoplasmic inclusions (a) and glial cytoplasmic inclusions (a’) of pTDP-43 (b). Normal controls have large Betz cells (Map 2+) located in layer 5 of the motor cortex with normal apical dendrites (arrows) with no evidence of microgliosis (Iba1+) or astrogliosis (GFAP+). Inset is enlarged bellow. c Graph represents percentage of normal microglia per section ± SEM in normal controls (black column) and with TDP-43 pathology (white column). d Graph represents percentage of activated microglia per section ± SEM in normal controls (black column) and withTDP-43 pathology (white column). e, f ALS cases with TDP-43 pathology have smaller Betz cells, microgliosis with rod-like microglia, and astrogliosis. g, h ALS-FTLD cases with TDP-43 pathology also have smaller Betz cells, microgliosis with rod-like microglia, and astrogliosis. Insets are enlarged bellow. ***p < 0.0001. Asterisk denotes normal microglia, arrows point to apical dendrites, arrowheads point to activated microglia, and number signs point to rod-like microglia. Scale bar = 20 μm