SQSTM1 and amyotrophic lateral sclerosis: Several years later, the TDP-43 protein (TAR DNA-binding protein 43, encoded by the gene TARDBP) was found as the most common component of ubiquitin-positive protein inclusions in ALS and FTD affected patient brains, together with the macroautophagy scaffold protein Sequestosome 1 (SQSTM1/p62; Neumann et al., 2006).