TARDBP and amyotrophic lateral sclerosis: More notably, cytoplasmic mislocalization, aggregation, and cleavage of TDP-43 (TDP-43 pathology) in motor neurons and glial cells have been found in ~97% of all ALS cases, and consequently regarded as a pathological hallmark of ALS (Neumann et al., 2006; Mackenzie et al., 2007).