Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells

Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells

Niemann-Pick C (NPC) disease is an autosomal recessive disorder that leads to excessive storage of cholesterol and other lipids in late endosomes and lysosomes. The large majority of NPC disease is caused by mutations in NPC1, a large polytopic membrane protein that functions in late endosomes. Ther...

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Bibliographic Details
Main Authors: Nina H. Pipalia, Kanagaraj Subramanian, Shu Mao, Harold Ralph, Darren M. Hutt, Samantha M. Scott, William E. Balch, Frederick R. Maxfield
Format: Article
Language:English
Published: Elsevier 2017-04-01
Series:Journal of Lipid Research
Subjects:
NPC1
cellular cholesterol
lipid transport
inborn errors of metabolism
drug therapy
cholesterol trafficking
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520338475

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http://www.sciencedirect.com/science/article/pii/S0022227520338475

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