TLR4 knockout in the SOD1G93A genetic background extended survival of mice by 2 weeks (Lee et al., 2015), while a prolonged pharmacological inhibition of TLR4 in SOD1G93A mice results in a mild attenuation of MNs degeneration but not in prolongation of survival (Fellner et al., 2017; Figure 2). The gene discussed is TLR4; the disease is Melnick-Needles syndrome.