Both TLR2 and TLR9 protein or mRNA levels are elevated in tissue samples from patients with neurodegenerative diseases including ALS, PD, and GBS [32, 33, 47–49] with patterns of increased inflammatory mediators in peripheral or CSF samples generally corresponding to the inflammatory mediators that are released upon activation of TLR2 and TLR9 (e.g., TNFα, IL-18, IL-1β, IL-6, IL-8, MCP-1, MIP-1α, IL-15, IL-17 IFN-ɣ, and MIP-1β in ALS and IL-1β, IL-2, IL-4, IL-6, IL-8, TGF-α, TGF-β, MCP-1, MIP-1α, and INF-ɣ in plasma or CSF samples from PD patients). Here, TLR2 is linked to amyotrophic lateral sclerosis.