Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration with TDP-43 pathology (FTLD-TDP) are the most prominent TDP-43 proteinopathies, with TDP-43 inclusions found in approximately 97% of ALS cases [2] and 32–54% of FTLD cases [3,4]. This evidence concerns the gene TARDBP and proteostasis deficiencies.