While heterozygous Tbk1 knock-out mice did not develop MN degeneration or autophagy impairments (Brenner et al. 2019), loss of murine Tbk1 resulted embryonic lethal (Bonnard et al. 2000), and animals undergoing neuron-specific Tbk1 deletion displayed loss of cortical synapses, dendrite dysmorphism, and accumulation of neurofibrillary tangles correlating with motor and cognitive defects reminiscent of ALS/FTD (Duan et al. 2019). Here, TBK1 is linked to frontotemporal dementia.