Although another Tdp-43 mouse model of ALS shows Tdp-43 mislocalization associated, this model involves Cre-lox-mediated deletion of exons 2 and 3 encoding the RNA-recognition motif (RRM) regions in addition to NLS sequence, resulting in human ALS irrelevant alternative splicing and RNA processing defects [28]. Here, TARDBP is linked to amyotrophic lateral sclerosis.