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Article

HSP90 inhibitors reduce cholesterol storage in Niemann-Pick type C1 mutant fibroblasts.

Journal of lipid research - 1 Jan 2021

Pipalia Nina H, Saad Syed Z, Subramanian Kanagaraj, Cross Abigail, Al-Motawa Aisha, Garg Kunal, Blagg Brian S J, Neckers Len, Helquist Paul, Wiest Olaf, Ory Daniel S, Maxfield Frederick R

Abstract excerpt

Niemann-Pick type C1 (NPC1) disease is a lysosomal lipid storage disorder caused by mutations of the NPC1 gene. More than 300 disease-associated mutations are reported in patients, resulting in abnormal accumulation of unesterified cholesterol, glycosphingolipids, and other lipids in late endosomes and lysosomes (LE/Ly) of many cell types. Previously, we showed that treatment of many different NPC1 mutant...

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Identifiers

PMID: 34481829
DOI: 10.1016/j.jlr.2021.100114
PMCID: PMC8517605

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