Besides TDP-43, another RNA-binding protein was found to be strongly implicated in the disease: mutations in fused in sarcoma (FUS) are found in ~5 % of ALS cases and rare cases of FTLD [60, 99, 123], and wild-type FUS protein aggregates are present in about 10 % of FTLD patients [89]. Here, TARDBP is linked to amyotrophic lateral sclerosis.