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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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Publicado en: | Orphanet J Rare Dis |
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Autores principales: | , , , |
Formato: | Artigo |
Lenguaje: | Inglês |
Publicado: |
BioMed Central
2017
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Materias: | |
Acceso en línea: | 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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