The detection of TDP-43-containing pathological aggregates in numerous neurodegenerative diseases—including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia—has resulted in the classification of a spectrum of disorders known as TDP-43 proteinopathies (Kwong et al., 2008; Ling et al., 2013). Here, TARDBP is linked to amyotrophic lateral sclerosis.