Through generation of a specific antibody, we have demonstrated the upregulation of sTDP-43 protein isoforms sharing the unique C-terminal sequence in lumbar spinal cord tissue from ALS cases versus controls, and that sTDP-43 isoform(s) are integrated in TDP-43 pathology present in spinal motor neurons of ALS cases. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.