Because nearly all sporadic ALS cases and more than half of sporadic FTLD cases have cytoplasmic inclusions consisting of the cleaved form of hyper-phosphorylated TDP-43 (Ling et al., 2013), TDP-43 proteinopathy was proposed as a concept that implicates FTLD-TDP and ALS as a continuous disease spectrum. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.