Additional studies suggest a role for TLR/RAGE (receptor for advanced glycation end-products) signaling in ALS, e.g., increased expression of TLR2, TLR4, and RAGE was found in the spinal cords of ALS patients [275]; however, it is unclear to which extent neuroinflammation contributes to neurodegeneration. Here, TLR4 is linked to amyotrophic lateral sclerosis.