Staging of TDP-43 pathology based on post-mortem immunohistochemical analysis of ALS brain and spinal cord has revealed the spatio-temporal spread of pathology from the initially affected region to other neuroanatomically connected brain regions.6,7 These data advocate for the presence of specific pathogenic forms of TDP-43 that can transmit from cell to cell and transform conformation of the native protein into pathogenic form in a prion-like mechanism. Here, TARDBP is linked to amyotrophic lateral sclerosis.