Article
Enrichment of NPC1-deficient cells with the lipid LBPA stimulates autophagy, improves lysosomal function, and reduces cholesterol storage.
The Journal of biological chemistry - 1 Jul 2021
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
Abstract excerpt
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 anionic lipid lysobisphosphatidic acid (LBPA; also called bis(monoacylglycerol)phosphate) via treatment with its...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.