It is not possible to discount, though, that the immune response and B-cell activation may be more relevant in subsets of ALS patients characterized by specific genotypes: recently, clonally expanded CD8+ T cells have been identified in familial ALS-4 cases (4), and autoimmune response characterize a murine model lacking C9Orf72 protein expression (51); furthermore, OCB appears to be disproportionately more common in ALS patients with TDP-43 mutations (28). Here, C9orf72 is linked to amyotrophic lateral sclerosis.