TLR2 expression in microglia was also enhanced in ALS mouse model, and the increased expression of TLR2 was strongly associated with aggravated neuroinflammation and degeneration of motor neurons.298 TLR2 expression was also enhanced in the post-mortem spinal cord tissue from sporadic ALS patients.299 Overexpression of mutant SOD1 enhanced the activation of microglia, which was mediated by TLR2.300. Here, SOD1 is linked to amyotrophic lateral sclerosis.