Transgenic mice with doxycycline (Dox)-suppressible expression of human TDP-43 containing a defective nuclear localization signal (hTDP-43∆NLS) under the control of the neurofilament heavy chain promoter (rNLS8 mice) are one of the most disease-relevant models of ALS, displaying progressive TDP-43 cytoplasmic accumulation coinciding with rapid ALS-like neurodegeneration and motor decline [18, 19]. Here, NEFH is linked to amyotrophic lateral sclerosis.