Fig. 3
From: From animal models to human disease: a genetic approach for personalized medicine in ALS
Neuropathological findings in human sALS cases and animal models of ALS. Microscopic pictures of neuropathological findings in ALS models. Our previously published TDP-43 and SOD1 mouse models were exploited for illustration of TDP-43 and SOD1 aggregates with permission. a Immunofluorescence microscopy of a hSOD1G93A mouse spinal cord. The B8H10 antibody was utilized for the specific signal of misfolded SOD1. Pictures were taken at 10x and b 40x magnification for better visualization of aggregates. c-e Double immunofluorescence microscopy of 10 months-old a hTDP-43G348C mouse spinal cord using hTDP-43 monoclonal antibody and d ubiquitin antibody [138]. Ubiquinated TDP-43 cytoplasmic aggregates can be observed and are typical neuropathological findings in human ALS. f-i Immunofluorescence of 10 months-old hTDP-43G348C and non-transgenic mice spinal cord. Iba1 antibody f-g and GFAP antibody h-i showed increased microgliosis and astrogliosis in a 10 months-old hTDP-43G348C mouse. j-l Immunohistochemistry of two human sporadic ALS cases using TDP-43 antibody to illustrated typical neuronal cytoplasmic TDP-43 inclusions in lumbar spinal cord (j), medulla (k) and motor cortex (l). Scale bar = 250 μm (a); 50 μm (b, f-i); 25 μm (c-e); 100 μm (j-l)