Over the last decade, RAGE has gained attention as a potential contributor to the pathogenesis of various neurodegenerative diseases and conditions, such as Alzheimer’s disease (AD), Parkinson’s disease, Huntington’s disease, Creutzfeldt-Jakob disease, diabetic neuropathy, familial amyloid polyneuropathy, Charcot neuroarthropathy, and vasculitic neuropathy (Juranek et al., 2015). Here, AGER is linked to Huntington disease.