Enhanced RAGE expression has been observed in other neurodegenerative disorders, such as AD (Sasaki et al., 2001; Wang et al., 2009) and Huntington’s disease (Ma and Nicholson, 2004; Anzilotti et al., 2012), thereby suggesting a potential role for RAGE in the pathogenesis of these diseases. This evidence concerns the gene AGER and juvenile Huntington disease.