snRNA-seq analysis of TDP-43 KI mice. Mus musculus

Point mutations in the TARDBP gene encoding TDP-43 have been identified in some familial cases of amyotrophic lateral sclerosis (ALS). To elucidate the disease mechanisms, we established a TDP-43 knock-in (KI) mouse model carrying an ALS-linked point mutation. TDP-43 KI mice develop the key features of ALS, including motor dysfunction, loss of spinal motor neurons and increased insolubility of TDP-43 in an age-dependent and mutant TDP-43-dose dependent manner. To investigate changing of cell composition in spinal cords and gene expression in spinal motor neurons, we performed single nucleus RNA-seq (snRNA-seq) analysis for spinal cord samples dissected from TDP-43 KI mice at 6 months and 1 year of age.