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

Abstract 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...

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Bibliographic Details
Main Authors: Franziska Peter, Sebastian Rost, Arndt Rolfs, Moritz J. Frech
Format: Article
Language:English
Published: BMC 2017-08-01
Series:Orphanet Journal of Rare Diseases
Subjects:
PKC
Online Access:http://link.springer.com/article/10.1186/s13023-017-0697-y