A recent case report of atypical FTLD family cases characterized by astrocyte-predominant tauopathy, an aberrant tau isoform ratio, and a Q140H substitution in the FUS gene (Ferrer et al., 2015) supports the notion of a pathophysiological link between FUS and tau in FTLD/ALS through the regulation of 4R-tau/3R-tau isoforms. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.