The therapeutic potential of attenuated TLR4 signalling in ALS is highlighted in studies demonstrating reduced motoneuron degeneration in SOD1G93A mice lacking tlr4 (Lee et al., 2015), and in rescued SOD1G93A stimulated motoneuron death in vitro using TLR4 inhibitors (De Paola et al., 2016). Here, TLR4 is linked to amyotrophic lateral sclerosis.