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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Bibliographic Details
Main Authors: Saha, Arjun, Kim, Sung-Jo, Zhang, Zhongjian, Lee, Yi-Ching, Sarkar, Chinmoy, Tsai, Pei-Chih, Mukherjee, Anil B.
Format: Artigo
Language:Inglês
Published: 2008
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Article
Online Access: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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