Mutations in other RNA-binding proteins, including hnRNPA1, hnPA2B1 and MATR3, and a hexanucleotide repeat expansion in the C9ORF72 gene also cause TDP-43 proteinopathy, suggesting that the interaction of these RNA-binding proteins with TDP-43 and disruption of RNA metabolic homeostasis are involved in the intracytoplasmic accumulation of TDP-43 [80, 154, 164, 243]. This evidence concerns the gene TARDBP and torsades de pointes.