Given the involvement of impaired protein homeostasis, ALS, similar to other neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease and Huntington’s disease, is viewed as a proteinopathy, with most cases characterized pathologically by the presence of TAR DNA-binding protein 43 (TDP43)-containing ubiquitinated inclusions (Neumann et al., 2006). This evidence concerns the gene TARDBP and Huntington disease.