Protein aggregates containing truncated hyperphosphorylated and/or ubiquitinated TDP-43 are found within MNs in >95% of ALS-affected brains and spinal cords (Chou et al., 2018), and can occur in other neurological disorders, including Alzheimer’s, Parkinson’s and Huntington’s diseases, highlighting the importance of TDP-43 in neurodegeneration (Liu et al., 2017; St-Amour et al., 2018). The gene discussed is TARDBP; the disease is juvenile Huntington disease.