ALS is histologically characterized by large variety of cytoplasmic inclusions in both neuronal and glial cells positive for transactivation response DNA-binding protein 43 kDa (TDP-43); this is a key feature for up to 97% of ALS cases [15,16], whereas others show FUS and/or tau reactivity (see [151,152]). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.