In both neurons and glia of these patients, TDP43 is mislocalized from the nucleus to the cytoplasm, where it accumulates in abnormal phosphorylated, ubiquitinated and proteolytically-cleaved aggregates in 97% of ALS and 45% of FTD patients (sporadic and familial cases combined) [334]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.