Enrichment of NPC1-deficient cells with the lipid LBPA stimulates autophagy, improves lysosomal function, and reduces cholesterol storage

Niemann–Pick C (NPC) is an autosomal recessive disorder characterized by mutations in the NPC1 or NPC2 genes encoding endolysosomal lipid transport proteins, leading to cholesterol accumulation and autophagy dysfunction. We have previously shown that enrichment of NPC1-deficient cells with the anion...

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Detalles Bibliográficos
Autores principales: Ilnytska, Olga, Lai, Kimberly, Gorshkov, Kirill, Schultz, Mark L., Tran, Bruce Nguyen, Jeziorek, Maciej, Kunkel, Thaddeus J., Azaria, Ruth D., McLoughlin, Hayley S., Waghalter, Miriam, Xu, Yang, Schlame, Michael, Altan-Bonnet, Nihal, Zheng, Wei, Lieberman, Andrew P., Dobrowolski, Radek, Storch, Judith
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2021
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8294588/
https://www.ncbi.nlm.nih.gov/pubmed/34023384
http://dx.doi.org/10.1016/j.jbc.2021.100813

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8294588/
https://www.ncbi.nlm.nih.gov/pubmed/34023384
http://dx.doi.org/10.1016/j.jbc.2021.100813

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