Importantly, another recent study in transgenic ALS/FTD mice expressing hTDP-43 mutants has shown that the mislocalized TDP-43 represses the translation of neurofilament mRNAs (NEFL, NEFM and INA), giving rise to a proteomic signature functionally associated with cytoskeletal disorganization [84]. Here, TARDBP is linked to amyotrophic lateral sclerosis.