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

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...

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Detalles Bibliográficos
Autores principales: 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.
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/PMC8517605/
https://www.ncbi.nlm.nih.gov/pubmed/34481829
http://dx.doi.org/10.1016/j.jlr.2021.100114

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