TARDBP and amyotrophic lateral sclerosis: Next, behavioral experiments (open-field and rotarod tests) assessed motor function and analyzed pathologies using morphological assessments.<h4>Results</h4>Our TIA1Δ mouse model phenocopies select pivotal features of ALS, including TAR DNA-binding protein 43 (TDP-43) accumulation, motor neuron loss, neuroinflammation in the lumbar spinal cord, and muscle atrophy.