TARDBP and muscular atrophy: Here, we show that the selective overexpression of mutant UBQLN2P497H in the spinal motor neurons led to age-dependent impairment of motor functions in ChATtTA/UBQLN2P497H rats, including motor neuron degeneration, skeletal muscle atrophy, progressive impairment of motor function, TDP-43 pathology, ubiquitination and glial reactions, and abnormal protein accumulation.