Aggregates of the RNA-binding protein TDP-43 are incorporated in ubiquitinated inclusions within the neuronal cytoplasm found in ALS and in syndromes jointly named ‘neurodegeneration with brain iron accumulation’ (NBIA) (Neumann et al., 2006; Haraguchi et al., 2011). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.