The Niemann-Pick C1 Gene Is Downregulated in Livers of C57BL/6J Mice by Dietary Fatty Acids, but Not Dietary Cholesterol, through Feedback Inhibition of the SREBP Pathway3

Garver, 2007, The National Niemann-Pick C1 disease database: report of clinical features and health problems., Am J Med Genet., 143A, 1204, 10.1002/ajmg.a.31735 Imrie, 2007, The natural history of Niemann-Pick disease type C in the UK., J Inherit Metab Dis., 30, 51, 10.1007/s10545-006-0384-7 Carstea, 1997, Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis., Science., 277, 228, 10.1126/science.277.5323.228 Davies, 2000, Transmembrane molecular pump activity of Niemann-Pick C1 protein., Science., 290, 2295, 10.1126/science.290.5500.2295 Davies, 2000, J Biol Chem., 275, 24367, 10.1074/jbc.M002184200 Infante, 2008, Purified NPC1 protein: II. Localization of sterol binding to a 240-amino acid soluble luminal loop., J Biol Chem., 283, 1064, 10.1074/jbc.M707944200 Ohgami, 2004, Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain., Proc Natl Acad Sci USA., 101, 12473, 10.1073/pnas.0405255101 Garver, 2000, Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments., J Lipid Res., 41, 673, 10.1016/S0022-2275(20)32376-2 Higgins, 1999, Niemann-Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the trans-Golgi network., Mol Genet Metab., 68, 1, 10.1006/mgme.1999.2882 Chen, 2005, NPC1 late endosomes contain elevated levels of non-esterified ('free') fatty acids and an abnormally glycosylated form of the NPC2 protein., Biochem J., 390, 549, 10.1042/BJ20050236 Liscum, 1989, The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts., J Cell Biol., 108, 1625, 10.1083/jcb.108.5.1625 Meyre, 2009, Genome-wide association study for early-onset and morbid adult obesity identifies three new risk loci in European populations., Nat Genet., 41, 157, 10.1038/ng.301 Hsu, 2000, Fine linkage and physical mapping suggests cross-over suppression with a retroposon insertion at the Npc1 mutation., Mamm Genome., 11, 774, 10.1007/s003350010134 Loftus, 1997, Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene., Science., 277, 232, 10.1126/science.277.5323.232 Jelinek, 2010, Decreased Npc1 gene dosage in mice is associated with weight gain., Obesity (Silver Spring)., 18, 1457, 10.1038/oby.2009.415 Jelinek, 2011, Npc1 haploinsufficiency promotes weight gain and metabolic features associated with insulin resistance., Hum Mol Genet., 20, 312, 10.1093/hmg/ddq466 Jelinek, 2011, The Niemann-Pick C1 gene interacts with a high-fat diet and modifying genes to promote weight gain., Am J Med Genet., 155A, 2317, 10.1002/ajmg.a.34135 Garver, 2005, Niemann-Pick C1 expression is not regulated by the amount of cholesterol flowing through cells in the mouse., J Lipid Res., 46, 1745, 10.1194/jlr.M500130-JLR200 Maxwell, 2003, Novel putative SREBP and LXR target genes identified by microarray analysis in liver of cholesterol-fed mice., J Lipid Res., 44, 2109, 10.1194/jlr.M300203-JLR200 Garver, 2008, The Niemann-Pick C1 gene is downregulated by feedback inhibition of the SREBP pathway in human fibroblasts., J Lipid Res., 49, 1090, 10.1194/jlr.M700555-JLR200 Gévry, 2008, Cholesterol supply and SREBPs modulate transcription of the Niemann-Pick C-1 gene in steroidogenic tissues., J Lipid Res., 49, 1024, 10.1194/jlr.M700554-JLR200 Alexander, 2006, Distinct phenotypes of obesity-prone AKR/J, DBA2J and C57BL/6J mice compared to control strains., Int J Obes (Lond)., 30, 50, 10.1038/sj.ijo.0803110 Rossmeisl, 2003, Variation in type 2 diabetes: related traits in mouse strains susceptible to diet-induced diabetes., Diabetes., 52, 1958, 10.2337/diabetes.52.8.1958 Shimomura, 1997, Differential expression of exons 1a and 1c in mRNAs for sterol regulatory element-binding protein-1 in human and mouse organs and cultured cells., J Clin Invest., 99, 838, 10.1172/JCI119247 Garver, 2007, Characterization of liver disease and lipid metabolism in the Niemann-Pick C1 mouse., J Cell Biochem., 101, 498, 10.1002/jcb.21200 Katz, 2000, Quantitative insulin sensitivity check index: a simple, accurate method for assessing insulin sensitivity in humans., J Clin Endocrinol Metab., 85, 2402, 10.1210/jcem.85.7.6661 Matthews, 1985, Homeostasis model assessment: insulin resistance and beta-cell function from fasting plasma glucose and insulin concentrations in man., Diabetologia., 28, 412, 10.1007/BF00280883 Nakav, 2009, Blocking adenosine A2A receptor reduces peritoneal fibrosis in two independent experimental models., Nephrol Dial Transplant., 24, 2392, 10.1093/ndt/gfp041 Bult, 2008, The Mouse Genome Database (MGD): mouse biology and model systems., Nucleic Acids Res., 36, D724, 10.1093/nar/gkm961 Gévry, 2002, The role and regulation of the Niemann-Pick C1 gene in adrenal steroidogenesis., Endocr Res., 28, 403, 10.1081/ERC-120016815 Amemiya-Kudo, 2002, Transcriptional activities of nuclear SREBP-1a, -1c, and -2 to different target promoters of lipogenic and cholesterogenic genes., J Lipid Res., 43, 1220, 10.1194/jlr.M100417-JLR200 Dietschy, 1998, Dietary fatty acids and the regulation of plasma low density lipoprotein cholesterol concentrations., J Nutr., 128, 444S, 10.1093/jn/128.2.444S Rumsey, 1995, Oleate and other long chain fatty acids stimulate low density lipoprotein receptor activity by enhancing acyl coenzyme A:cholesterol acyltransferase activity and altering intracellular regulatory cholesterol pools in cultures cells., Proc Natl Acad Sci USA., 270, 10008 Worgall, 1998, Polyunsaturated fatty acids decrease expression of promoters with sterol regulatory elements by decreasing levels of mature sterol regulatory element-binding protein., J Biol Chem., 273, 25537, 10.1074/jbc.273.40.25537 Ou, 2001, Unsaturated fatty acids inhibit transcription of the sterol regulatory element-binding protein-1c (SREBP-1c) gene by antagonizing ligand-dependent activation of LXR., Proc Natl Acad Sci USA., 98, 6027, 10.1073/pnas.111138698 Yoshikawa, 2002, Polyunsaturated fatty acids suppress sterol regulatory element-binding protein 1c promoter activity by inhibition of liver X receptor (LXR) binding to LXR response elements., J Biol Chem., 277, 1705, 10.1074/jbc.M105711200 Lee, 2010, Identification of Ubxd8 protein as a sensor for unsaturated fatty acids and regulator of triglyceride synthesis., Proc Natl Acad Sci USA., 107, 21424, 10.1073/pnas.1011859107 Shimomura, 1997, Cholesterol feeding reduces nuclear forms of sterol regulatory element-binding proteins in hamster liver., Proc Natl Acad Sci USA., 94, 12354, 10.1073/pnas.94.23.12354 Amemiya-Kudo, 2000, Promoter analysis of the mouse sterol regulatory element-binding protein-1c gene., J Biol Chem., 275, 31078, 10.1074/jbc.M005353200 Takeuchi, 2010, Polyunsaturated fatty acids selectively suppress sterol regulatory element-binding protein-1 through proteolytic processing and autoloop regulatory circuit., J Biol Chem., 285, 11681, 10.1074/jbc.M109.096107 Sato, 1996, Sterol-dependent transcriptional regulation of sterol regulatory element-binding protein-2., J Biol Chem., 271, 26461, 10.1074/jbc.271.43.26461 Ohsaki, 2006, Cholesterol depletion facilitates ubiquitylation of NPC1 and its association with SKD1/Vps4., J Cell Sci., 119, 2643, 10.1242/jcs.02993