FTD and ALS exhibit common molecular pathological features, including the mislocalization and aggregation of TAR DNA-binding protein 43 (TDP-43), a ribonucleotide protein that regulates mRNA metabolism, the accumulation of FTD/ALS-associated mutated proteins in inclusions, and the failure of the PQC system [173, 176, 177]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.