Mutations in TARDBP account for a small percentage (< 5%) of ALS cases; however, cytoplasmic TDP‐43 inclusions, accompanied by nuclear depletion of the protein, are the key pathological hallmark of > 98% of ALS indicating the profound importance of TDP‐43 in pathogenesis (Mackenzie et al, 2010; Sreedharan et al, 2008); furthermore, TDP‐43 deposition has been consistently described in a range of other neurodegenerative diseases most prominently frontotemporal dementia (Neumann et al, 2006). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.