TARDBP and amyotrophic lateral sclerosis: In familial cases, mutations in over 20 genes have been associated with ALS, including TAR DNA binding protein (TARDBP) that encode TDP-43.4 Currently, there is no cure for ALS and the FDA-approved drug Riluzole extends survival, albeit only by ∼3 months.5 Although the exact mechanism by which Riluzole improves survival is unclear, it appears to reduce glutamate release, attenuate persistent sodium and inward calcium currents,6,7 whilst at the same time leading to an improvement in functional capacity of mitochondria.8