TARDBP and amyotrophic lateral sclerosis: TDP-43 has been reproducibly identified as one of the components of cytosolic protein inclusions, a pathological hallmark in almost all ALS and some FTD cases (known pathologically as frontotemporal lobar degeneration with TDP-43 pathology, FTLD-TDP), irrespective of genetic inheritance or mutation (Neumann et al., 2006, 2007; Wang et al., 2008).