Non-genetically modified models exhibit TARDBP mRNA increase due to perturbed TDP-43 autoregulation
Amyotrophic lateral sclerosis (ALS) is a here neurodegenerative disease characterized by accumulation of fragmented insoluble TDP-43 and loss of TDP-43 from the nucleus.Increased expression of exogenous TARDBP (encoding TDP-43) induces TDP-43 pathology and cytotoxicity, suggesting the involvement of aberrant expression of TDP-43 in the pathogenesis