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ER\u2010stress disrupts Ca<jats:sup>2+<\/jats:sup>\u2010homeostasis and stimulates the expression of Ca<jats:sup>2+<\/jats:sup>\u2010binding proteins. We report here that in the PPT1\u2010deficient human and mouse brain the levels of S100B, a Ca<jats:sup>2+<\/jats:sup>\u2010binding protein, and its receptor, RAGE (receptor for advanced glycation end\u2010products) are elevated. We further demonstrate that activation of RAGE signaling in astroglial cells mediates pro\u2010inflammatory cytokine production, which is inhibited by SiRNA\u2010mediated suppression of RAGE expression. 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