Furthermore, in 2006 TDP-43 was identified as the major inclusion protein in thiscondition and is associated with UI inclusions in the vast majority of ALS patientsas well as in the most common pathological subtype of FTD, now referred to as FTLDwith TDP-43 pathology.19,29 Recognition of this mutation inTDP-43 as being causal to ALS and FTD quickly led to screeningfor other RNA binding proteins. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.