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Quantitative Comparison of the Efficacy of Various Compounds in Lowering Intracellular Cholesterol Levels in Niemann-Pick Type C Fibroblasts
Niemann-Pick Type C disease (NPC) is a lethal, autosomal recessive disorder caused by mutations in the NPC1 and NPC2 cholesterol transport proteins. NPC’s hallmark symptoms include an accumulation of unesterified cholesterol and other lipids in the late endosomal and lysosomal cellular compartments,...
Autores principales: | Wehrmann, Zachary T., Hulett, Tyler W., Huegel, Kara L., Vaughan, Kevin T., Wiest, Olaf, Helquist, Paul, Goodson, Holly |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3483179/ https://www.ncbi.nlm.nih.gov/pubmed/23144769 http://dx.doi.org/10.1371/journal.pone.0048561 |
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