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RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid lipofuscinosis
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(++)-homeost...
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Hauptverfasser: | , , , , , , |
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Format: | Artigo |
Sprache: | Inglês |
Veröffentlicht: |
2008
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Schlagworte: | |
Online Zugang: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2597448/ https://ncbi.nlm.nih.gov/pubmed/18948101 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.febslet.2008.10.015 |
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