Thereby, we were able to generate two different types of murine Tdp-43ΔNLS mice models of ALS: one with inducible Cre-mediated Tdp-43ΔNLS expression and the other with developmentally regulated Mnx1-driven Cre expression-mediated Tdp-43ΔNLS in post-mitotic neurons in the brain. Here, MNX1 is linked to amyotrophic lateral sclerosis.