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Cholesterol binding to the sterol-sensing region of Niemann Pick C1 protein confines dynamics of its N-terminal domain

Lysosomal accumulation of cholesterol is a hallmark of Niemann Pick type C (NPC) disease caused by mutations primarily in the lysosomal membrane protein NPC1. NPC1 contains a transmembrane sterol-sensing domain (SSD), which is supposed to regulate protein activity upon cholesterol binding, but the m...

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Detalles Bibliográficos
Autores principales: Dubey, Vikas, Bozorg, Behruz, Wüstner, Daniel, Khandelia, Himanshu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7537887/
https://www.ncbi.nlm.nih.gov/pubmed/33021976
http://dx.doi.org/10.1371/journal.pcbi.1007554