Thirdly, the amount of the phosphorylated TDP-43 (pTDP-43), which was identified as a component of the ubiquitin-positive inclusions (UPIs) in diseased neurons of ALS and FTLD [48], was increased significantly in the spinal cord (see Additional file 4: Figure S4a), but not the forebrain (see Additional file 4: Figure S4b), of mice at the age of 12 months and beyond. Here, TARDBP is linked to amyotrophic lateral sclerosis.