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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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Détails bibliographiques
Publié dans:Orphanet J Rare Dis
Auteurs principaux: Peter, Franziska, Rost, Sebastian, Rolfs, Arndt, Frech, Moritz J.
Format: Artigo
Langue:Inglês
Publié: BioMed Central 2017
Sujets:
Accès en ligne: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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