Endosomal Accumulation of Toll-Like Receptor 4 Causes Constitutive Secretion of Cytokines and Activation of Signal Transducers and Activators of Transcription in Niemann–Pick Disease Type C (NPC) Fibroblasts: A Potential Basis for Glial Cell Activation in the NPC Brain

Niemann–Pick disease type C (NPC) is an inherited lipid storage disorder caused by mutations in NPC1 or NPC2 genes. Loss of function of either protein results in the endosomal accumulation of cholesterol and other lipids, progressive neurodegeneration, and robust glial cell activation. Here, we repo...

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Veröffentlicht in:J Neurosci
Hauptverfasser: Suzuki, Michitaka, Sugimoto, Yuko, Ohsaki, Yuki, Ueno, Makoto, Kato, Shinsuke, Kitamura, Yukisato, Hosokawa, Hiroshi, Davies, Joanna P., Ioannou, Yiannis A., Vanier, Marie T., Ohno, Kousaku, Ninomiya, Haruaki
Format: Artigo
Sprache:Inglês
Veröffentlicht: Society for Neuroscience 2007
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Articles
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6673540/
https://ncbi.nlm.nih.gov/pubmed/17314284
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.5282-06.2007
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