Although both mutant SOD1 and mutant TDP-43 reproduce typical ALS features in rodent models, overexpression of mutant SOD1 in spinal motor neurons does not lead to motor neuron death [29, 39], whereas selective expression of mutant TDP-43 in motor neurons causes substantial motor neuron death [16] in rats. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.