| First Author | Saha A | Year | 2008 |
| Journal | FEBS Lett | Volume | 582 |
| Issue | 27 | Pages | 3823-31 |
| PubMed ID | 18948101 | Mgi Jnum | J:140706 |
| Mgi Id | MGI:3814457 | Doi | 10.1016/j.febslet.2008.10.015 |
| Citation | Saha A, et al. (2008) RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid lipofuscinosis. FEBS Lett 582(27):3823-31 |
| abstractText | Palmitoyl-protein thioesterase-1 (PPT1) deficiency causes infantile neuronal ceroid lipofuscinosis (INCL), a devastating childhood neurodegenerative storage disorder. We previously reported that neuronal apoptosis in INCL is mediated by endoplasmic reticulum-stress. ER-stress disrupts Ca(2+)-homeostasis and stimulates the expression of Ca(2+)-binding proteins. We report here that in the PPT1-deficient human and mouse brain the levels of S100B, a Ca(2+)-binding protein, and its receptor, RAGE (receptor for advanced glycation end-products) are elevated. We further demonstrate that activation of RAGE signaling in astroglial cells mediates pro-inflammatory cytokine production, which is inhibited by SiRNA-mediated suppression of RAGE expression. We propose that RAGE signaling contributes to neuroinflammation in INCL. |
