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Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD

Niemann–Pick type C disease is a fatal, progressive neurodegenerative disorder caused by loss-of-function mutations in NPC1, a multipass transmembrane glycoprotein essential for intracellular lipid trafficking. We sought to define the cellular machinery controlling degradation of the most common dis...

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Detalles Bibliográficos
Autores principales: Schultz, Mark L., Krus, Kelsey L., Kaushik, Susmita, Dang, Derek, Chopra, Ravi, Qi, Ling, Shakkottai, Vikram G., Cuervo, Ana Maria, Lieberman, Andrew P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131187/
https://www.ncbi.nlm.nih.gov/pubmed/30202070
http://dx.doi.org/10.1038/s41467-018-06115-2