TARDBP and amyotrophic lateral sclerosis: This neuron-specific expression of TDP-43 leads to a very fast-progressing ALS model, with homozygous hTDP-43 mice showing a clinical phenotype of progressive hindlimb paralysis, body weight loss and a life expectancy of ∼20-25 days with TDP-43 pathology and motor neuron cell death in the spinal cord.42