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Activation of PKC triggers rescue of NPC1 patient specific iPSC derived glial cells from gliosis

BACKGROUND: Niemann-Pick disease Type C1 (NPC1) is a rare progressive neurodegenerative disorder caused by mutations in the NPC1 gene. The pathological mechanisms, underlying NPC1 are not yet completely understood. Especially the contribution of glial cells and gliosis to the progression of NPC1, ar...

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Pubblicato in:Orphanet J Rare Dis
Autori principali: Peter, Franziska, Rost, Sebastian, Rolfs, Arndt, Frech, Moritz J.
Natura: Artigo
Lingua:Inglês
Pubblicazione: BioMed Central 2017
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC5574080/
https://ncbi.nlm.nih.gov/pubmed/28841900
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13023-017-0697-y
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