Also in line with the prion-like hypothesis, the phosphorylated 43 kDa TAR DNA-binding protein (pTDP-43) has been identified as a major neuropathologic factor in ALS and frontotemporal lobar degeneration (Neumann et al., 2006; Geser et al., 2009). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.