In particular, P2X7 was found increased in activated microglia of ALS patients [58] and its selective activation by the agonist 2′-3′-O-(benzoyl-benzoyl) ATP (BzATP) in primary microglia isolated from SOD1-G93A mouse brain induces morphologic transition, enhances the production of proinflammatory mediators such as TNFα, and influences cyclooxygenase-2 (COX2), NOX2, Rac1, ERK1/2, p38, and NF-kB activation [15, 16, 19]. Here, TNF is linked to amyotrophic lateral sclerosis.