TARDBP and amyotrophic lateral sclerosis: Notably, mutations in the RNA-binding proteins (RBPs) TAR DNA-binding protein 43 (TDP43) and Fused in Sarcoma (FUS) are known causes of ALS, and pathologic TDP43 inclusions are found in the brain and spinal cord of 97% of ALS patients, suggesting that defects in RNA metabolism are a common feature in ALS.