Advanced glycation end products (AGE) and their receptor (RAGE) in the brain of patients with Creutzfeldt-Jakob disease with prion plaques.

The objective of this study was to assess the pathological role of advanced glycation end products (AGE) and the receptor for AGE (RAGE) in Creutzfeldt-Jakob disease (CJD). We immunohistochemically investigated the occipital lobe of three patients with CJD containing with prion protein (PrP) plaques using anti-AGE and RAGE antibodies. Many PrP-positive plaques were observed in these patients, and the PrP-positive prion plaques also showed immunoreactivity for the anti-AGE antibody. Furthermore, many astrocytes contained-PrP positive granules, and the same astrocytes also contained many AGE- and RAGE-immunopositive granules. The staining pattern of these granules showed good concordance with that of PrP. These findings suggest that there may be a RAGE-mediated PrP degradation pathway in CJD as is the case for beta-amyloid protein in Alzheimer's disease.