Histone deacetylase inhibitor treatment dramatically reduces cholesterol accumulation in Niemann-Pick type C1 mutant human fibroblasts

Niemann-Pick type C (NPC) disease is predominantly caused by mutations in the NPC1 protein that affect intracellular cholesterol trafficking and cause accumulation of unesterified cholesterol and other lipids in lysosomal storage organelles. We report the use of a series of small molecule histone de...

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Главные авторы: Pipalia, Nina H., Cosner, Casey C., Huang, Amy, Chatterjee, Anamitra, Bourbon, Pauline, Farley, Nathan, Helquist, Paul, Wiest, Olaf, Maxfield, Frederick R.
Формат: Artigo
Язык:Inglês
Опубликовано: National Academy of Sciences 2011
Предметы:
Biological Sciences
Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC3078401/
https://ncbi.nlm.nih.gov/pubmed/21436030
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1014890108
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