Spinal cord sections of transgenic SOD1G93A mice (a-c, f) show anterior horn cell degeneration, hyaline aggregates (arrow) and vacuolation (a) not present in corresponding wild-type (d). Amorphous cytoplasmic aggregates are ubiquitinated and occasionally extend into proximal processes (b, c). TDP-43 remains located in the nucleus of anterior horn cells and shows no distinct aggregates (f). The appearances are essentially indistinguishable from wild-type (e) apart from possible slightly weaker TDP-43 signal in presumed reactive glia (f). Samples from transgenic Smn-/-;SMN2;SMNΔ7 spinal cord (g-i) show residual motor neurons (g) without ubiquitin positivity (h) and preserved nuclear TDP-43 signal (i). Scale bar in all images = 20 μm.
Turner et al. BMC Neuroscience 2008 9:104 doi:10.1186/1471-2202-9-104