Article
Histone deacetylase inhibitor treatment dramatically reduces cholesterol accumulation in Niemann-Pick type C1 mutant human fibroblasts.
Proceedings of the National Academy of Sciences of the United States of America - 5 Apr 2011
Pipalia Nina H, Cosner Casey C, Huang Amy, Chatterjee Anamitra, Bourbon Pauline, Farley Nathan, Helquist Paul, Wiest Olaf, Maxfield Frederick R
Abstract excerpt
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 deacetylase (HDAC) inhibitors in tissue culture models of NPC human fibroblasts. Some HDAC inhibitors lead to a...
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