Several ALS/FTD mouse models of TDP-43 report that expression of ALS-linked mutations, cause brain atrophy, muscle denervation, motor neuron loss, and denervation of NMJs along with progressive motor impairments (McGoldrick et al., 2013; Picher-Martel et al., 2016; White et al., 2018; Ebstein et al., 2019; Huang et al., 2020; Alhindi et al., 2021). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.