The pathomechanisms resulting in abnormal mislocalisation and aggregation of TDP-43 within the CNS in ALS and FTD remain unknown, however there is a growing body of evidence to support an important role of neuroinflammation and innate immune-mediated mechanisms underlying the pathogenesis of neurodegeneration as recently reviewed for both diseases respectively [6,7]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.