A neuropathological cell model derived from Niemann−Pick disease type C patient-specific iPSCs shows disruption of the p62/SQSTM1−KEAP1−NRF2 Axis and impaired formation of neuronal networks

Niemann−Pick disease type C (NPC) is a rare neurodegenerative disorder caused by a recessive mutation in the NPC1 or NPC2 gene, in which patients exhibit lysosomal accumulation of unesterified cholesterol and glycolipids. Most of the research on NPC has been done in patient-derived skin fibroblasts...

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Detalles Bibliográficos
Autores principales: Saito, Ryo, Miyajima, Takashi, Iwamoto, Takeo, Wu, Chen, Suzuki, Ken, Hossain, Mohammad Arif, Munakata, Miyo, Era, Takumi, Eto, Yoshikatsu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8327345/
https://www.ncbi.nlm.nih.gov/pubmed/34377675
http://dx.doi.org/10.1016/j.ymgmr.2021.100784

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