Co-immunostaining of brain and spinal cord tissue sections with anti-TDP-43 and anti-NeuN revealed co-localization of both TDP-43 and NeuN in the cytosolic protein aggregates in the inner layers (III-V) of the motor cortex (Fig. 4a) and thoracic region of the spinal cord (Fig. 4b) of mutant mice but not in sham mice, suggesting that the observed ALS-like phenotypes in MN-specific Tdp-43ΔNLS mice were primarily due to Tdp-43 pathology in Mnx1-positive motor neurons in the CNS. The gene discussed is RBFOX3; the disease is amyotrophic lateral sclerosis.