RAGE binds a diverse set of ligands, particularly those noted as damage associated molecular patterns (DAMPs), including several upregulated in ALS patient and SOD1G93A mouse spinal cord, such as S100 calcium binding protein B, carboxymethyllysine (CML)-advanced glycation end product (AGE), and high-mobility group box 1 (HMGB1) [25, 27–29]. Here, HMGB1 is linked to amyotrophic lateral sclerosis.