Abnormal neuronal and glial inclusions composed of the transactive response DNA/RNA-binding protein Mr 43 kD (TDP-43) are the hallmark pathological lesions in two devastating neurodegenerative diseases subsumed as primary TDP-43 proteinopathies, frontotemporal lobar degeneration with TDP-43 pathology (FTLD–TDP), and in the vast majority of cases of amyotrophic lateral sclerosis (ALS–TDP) [1, 17]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.