Published in J Lipid Res on February 02, 2011
Study of Pharmacokinetics and Preliminary Efficacy in Patients With Niemann-Pick C1 | NCT02912793
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Ontogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease. Biochim Biophys Acta (2013) 0.79
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Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse. J Neurosci (2007) 1.71
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Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life. Pediatr Res (2010) 1.59
Expression of ABCG5 and ABCG8 is required for regulation of biliary cholesterol secretion. J Biol Chem (2004) 1.57
Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease. J Lipid Res (2007) 1.54
Cholesterol accumulation and liver cell death in mice with Niemann-Pick type C disease. Hepatology (2005) 1.48
Quantitation of two pathways for cholesterol excretion from the brain in normal mice and mice with neurodegeneration. J Lipid Res (2003) 1.45
Delineation of molecular changes in intrahepatic cholesterol metabolism resulting from diminished cholesterol absorption. J Lipid Res (2005) 1.42
Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease. J Neuropathol Exp Neurol (2005) 1.39
Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment. J Neurosci (2011) 1.33
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Receptor-mediated and bulk-phase endocytosis cause macrophage and cholesterol accumulation in Niemann-Pick C disease. J Lipid Res (2007) 1.21
ABCA1 plays no role in the centripetal movement of cholesterol from peripheral tissues to the liver and intestine in the mouse. J Lipid Res (2009) 1.14
HIF-2alpha-haploinsufficient mice have blunted retinal neovascularization due to impaired expression of a proangiogenic gene battery. Invest Ophthalmol Vis Sci (2008) 1.14
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Fenofibrate reduces intestinal cholesterol absorption via PPARalpha-dependent modulation of NPC1L1 expression in mouse. J Lipid Res (2007) 1.11
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Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream. J Lipid Res (2012) 1.07
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Multiple mechanisms limit the accumulation of unesterified cholesterol in the small intestine of mice deficient in both ACAT2 and ABCA1. Am J Physiol Gastrointest Liver Physiol (2010) 1.02
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Caveolin-1 is not required for murine intestinal cholesterol transport. J Biol Chem (2005) 0.99
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GM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 mice. J Lipid Res (2008) 0.91
Inhibition of cholesterol absorption by SCH 58053 in the mouse is not mediated via changes in the expression of mRNA for ABCA1, ABCG5, or ABCG8 in the enterocyte. J Lipid Res (2002) 0.91
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Cholesterol substrate pools and steroid hormone levels are normal in the face of mutational inactivation of NPC1 protein. J Lipid Res (2006) 0.88
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Niemann-Pick C1 expression is not regulated by the amount of cholesterol flowing through cells in the mouse. J Lipid Res (2005) 0.87
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