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Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann-Pick C1 Disease.

Lopez AM, Ramirez CM, Taylor AM, Jones RD, Repa JJ, Turley SD.

Dig Dis Sci. 2019 Jul 17. doi: 10.1007/s10620-019-05736-x. [Epub ahead of print]


ACSS2 promotes systemic fat storage and utilization through selective regulation of genes involved in lipid metabolism.

Huang Z, Zhang M, Plec AA, Estill SJ, Cai L, Repa JJ, McKnight SL, Tu BP.

Proc Natl Acad Sci U S A. 2018 Oct 2;115(40):E9499-E9506. doi: 10.1073/pnas.1806635115. Epub 2018 Sep 18.


1,25-Dihydroxyvitamin D3 enhances glucose-stimulated insulin secretion in mouse and human islets: a role for transcriptional regulation of voltage-gated calcium channels by the vitamin D receptor.

Kjalarsdottir L, Tersey SA, Vishwanath M, Chuang JC, Posner BA, Mirmira RG, Repa JJ.

J Steroid Biochem Mol Biol. 2019 Jan;185:17-26. doi: 10.1016/j.jsbmb.2018.07.004. Epub 2018 Jul 30.


Niemann-Pick C1-deficient mice lacking sterol O-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function.

Lopez AM, Jones RD, Repa JJ, Turley SD.

Am J Physiol Gastrointest Liver Physiol. 2018 Oct 1;315(4):G454-G463. doi: 10.1152/ajpgi.00124.2018. Epub 2018 Jun 7.


Normalization of Hepatic Homeostasis in the Npc1nmf164 Mouse Model of Niemann-Pick Type C Disease Treated with the Histone Deacetylase Inhibitor Vorinostat.

Munkacsi AB, Hammond N, Schneider RT, Senanayake DS, Higaki K, Lagutin K, Bloor SJ, Ory DS, Maue RA, Chen FW, Hernandez-Ono A, Dahlson N, Repa JJ, Ginsberg HN, Ioannou YA, Sturley SL.

J Biol Chem. 2017 Mar 17;292(11):4395-4410. doi: 10.1074/jbc.M116.770578. Epub 2016 Dec 28.


Impact of physiological levels of chenodeoxycholic acid supplementation on intestinal and hepatic bile acid and cholesterol metabolism in Cyp7a1-deficient mice.

Jones RD, Lopez AM, Tong EY, Posey KS, Chuang JC, Repa JJ, Turley SD.

Steroids. 2015 Jan;93:87-95. doi: 10.1016/j.steroids.2014.11.002. Epub 2014 Nov 28.


Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient mice.

Aqul A, Lopez AM, Posey KS, Taylor AM, Repa JJ, Burns DK, Turley SD.

Am J Physiol Gastrointest Liver Physiol. 2014 Oct 15;307(8):G836-47. doi: 10.1152/ajpgi.00243.2014. Epub 2014 Aug 21.


Histone deacetylase 9 represses cholesterol efflux and alternatively activated macrophages in atherosclerosis development.

Cao Q, Rong S, Repa JJ, St Clair R, Parks JS, Mishra N.

Arterioscler Thromb Vasc Biol. 2014 Sep;34(9):1871-9. doi: 10.1161/ATVBAHA.114.303393. Epub 2014 Jul 17.


Hearing loss is an early consequence of Npc1 gene deletion in the mouse model of Niemann-Pick disease, type C.

King KA, Gordon-Salant S, Pawlowski KS, Taylor AM, Griffith AJ, Houser A, Kurima K, Wassif CA, Wright CG, Porter FD, Repa JJ, Brewer CC.

J Assoc Res Otolaryngol. 2014 Aug;15(4):529-41. doi: 10.1007/s10162-014-0459-7. Epub 2014 May 17.


Sustained and selective suppression of intestinal cholesterol synthesis by Ro 48-8071, an inhibitor of 2,3-oxidosqualene:lanosterol cyclase, in the BALB/c mouse.

Chuang JC, Valasek MA, Lopez AM, Posey KS, Repa JJ, Turley SD.

Biochem Pharmacol. 2014 Apr 1;88(3):351-63. doi: 10.1016/j.bcp.2014.01.031. Epub 2014 Jan 31.


A functional, genome-wide evaluation of liposensitive yeast identifies the "ARE2 required for viability" (ARV1) gene product as a major component of eukaryotic fatty acid resistance.

Ruggles KV, Garbarino J, Liu Y, Moon J, Schneider K, Henneberry A, Billheimer J, Millar JS, Marchadier D, Valasek MA, Joblin-Mills A, Gulati S, Munkacsi AB, Repa JJ, Rader D, Sturley SL.

J Biol Chem. 2014 Feb 14;289(7):4417-31. doi: 10.1074/jbc.M113.515197. Epub 2013 Nov 22.


Role of calcium and EPAC in norepinephrine-induced ghrelin secretion.

Mani BK, Chuang JC, Kjalarsdottir L, Sakata I, Walker AK, Kuperman A, Osborne-Lawrence S, Repa JJ, Zigman JM.

Endocrinology. 2014 Jan;155(1):98-107. doi: 10.1210/en.2013-1691. Epub 2013 Dec 20.


The Xbp1s/GalE axis links ER stress to postprandial hepatic metabolism.

Deng Y, Wang ZV, Tao C, Gao N, Holland WL, Ferdous A, Repa JJ, Liang G, Ye J, Lehrman MA, Hill JA, Horton JD, Scherer PE.

J Clin Invest. 2013 Jan;123(1):455-68. doi: 10.1172/JCI62819. Epub 2012 Dec 21.


Carboxylesterases are uniquely expressed among tissues and regulated by nuclear hormone receptors in the mouse.

Jones RD, Taylor AM, Tong EY, Repa JJ.

Drug Metab Dispos. 2013 Jan;41(1):40-9. doi: 10.1124/dmd.112.048397. Epub 2012 Sep 25.


Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream.

Taylor AM, Liu B, Mari Y, Liu B, Repa JJ.

J Lipid Res. 2012 Nov;53(11):2331-42. doi: 10.1194/jlr.M028241. Epub 2012 Aug 14.


Delineation of biochemical, molecular, and physiological changes accompanying bile acid pool size restoration in Cyp7a1(-/-) mice fed low levels of cholic acid.

Jones RD, Repa JJ, Russell DW, Dietschy JM, Turley SD.

Am J Physiol Gastrointest Liver Physiol. 2012 Jul 15;303(2):G263-74. doi: 10.1152/ajpgi.00111.2012. Epub 2012 May 24.


Ghrelin directly stimulates glucagon secretion from pancreatic alpha-cells.

Chuang JC, Sakata I, Kohno D, Perello M, Osborne-Lawrence S, Repa JJ, Zigman JM.

Mol Endocrinol. 2011 Sep;25(9):1600-11. doi: 10.1210/me.2011-1001. Epub 2011 Jun 30.


Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment.

Aqul A, Liu B, Ramirez CM, Pieper AA, Estill SJ, Burns DK, Liu B, Repa JJ, Turley SD, Dietschy JM.

J Neurosci. 2011 Jun 22;31(25):9404-13. doi: 10.1523/JNEUROSCI.1317-11.2011.


Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations.

Ramirez CM, Liu B, Aqul A, Taylor AM, Repa JJ, Turley SD, Dietschy JM.

J Lipid Res. 2011 Apr;52(4):688-98. doi: 10.1194/jlr.M013789. Epub 2011 Feb 2.


Multiple mechanisms limit the accumulation of unesterified cholesterol in the small intestine of mice deficient in both ACAT2 and ABCA1.

Turley SD, Valasek MA, Repa JJ, Dietschy JM.

Am J Physiol Gastrointest Liver Physiol. 2010 Nov;299(5):G1012-22. doi: 10.1152/ajpgi.00190.2010. Epub 2010 Aug 19.


Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life.

Ramirez CM, Liu B, Taylor AM, Repa JJ, Burns DK, Weinberg AG, Turley SD, Dietschy JM.

Pediatr Res. 2010 Oct;68(4):309-15. doi: 10.1203/00006450-201011001-00604.


Chronic social defeat stress disrupts regulation of lipid synthesis.

Chuang JC, Cui H, Mason BL, Mahgoub M, Bookout AL, Yu HG, Perello M, Elmquist JK, Repa JJ, Zigman JM, Lutter M.

J Lipid Res. 2010 Jun;51(6):1344-53. doi: 10.1194/jlr.M002196. Epub 2010 Feb 2.


Liver X receptor agonists augment human islet function through activation of anaplerotic pathways and glycerolipid/free fatty acid cycling.

Ogihara T, Chuang JC, Vestermark GL, Garmey JC, Ketchum RJ, Huang X, Brayman KL, Thorner MO, Repa JJ, Mirmira RG, Evans-Molina C.

J Biol Chem. 2010 Feb 19;285(8):5392-404. doi: 10.1074/jbc.M109.064659. Epub 2009 Dec 11.


Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid.

Liu B, Ramirez CM, Miller AM, Repa JJ, Turley SD, Dietschy JM.

J Lipid Res. 2010 May;51(5):933-44. doi: 10.1194/jlr.M000257. Epub 2009 Nov 18.


Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse.

Liu B, Turley SD, Burns DK, Miller AM, Repa JJ, Dietschy JM.

Proc Natl Acad Sci U S A. 2009 Feb 17;106(7):2377-82. doi: 10.1073/pnas.0810895106. Epub 2009 Jan 26.


Inhibiting intestinal NPC1L1 activity prevents diet-induced increase in biliary cholesterol in Golden Syrian hamsters.

Valasek MA, Repa JJ, Quan G, Dietschy JM, Turley SD.

Am J Physiol Gastrointest Liver Physiol. 2008 Oct;295(4):G813-22. doi: 10.1152/ajpgi.90372.2008. Epub 2008 Aug 21.


Research resource: nuclear hormone receptor expression in the endocrine pancreas.

Chuang JC, Cha JY, Garmey JC, Mirmira RG, Repa JJ.

Mol Endocrinol. 2008 Oct;22(10):2353-63. doi: 10.1210/me.2007-0568. Epub 2008 Jul 31.


Regulation of nuclear import/export of carbohydrate response element-binding protein (ChREBP): interaction of an alpha-helix of ChREBP with the 14-3-3 proteins and regulation by phosphorylation.

Sakiyama H, Wynn RM, Lee WR, Fukasawa M, Mizuguchi H, Gardner KH, Repa JJ, Uyeda K.

J Biol Chem. 2008 Sep 5;283(36):24899-908. doi: 10.1074/jbc.M804308200. Epub 2008 Jul 7.


GM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 mice.

Li H, Turley SD, Liu B, Repa JJ, Dietschy JM.

J Lipid Res. 2008 Aug;49(8):1816-28. doi: 10.1194/jlr.M800180-JLR200. Epub 2008 Apr 30.


Impaired insulin secretion and glucose intolerance in synaptotagmin-7 null mutant mice.

Gustavsson N, Lao Y, Maximov A, Chuang JC, Kostromina E, Repa JJ, Li C, Radda GK, S├╝dhof TC, Han W.

Proc Natl Acad Sci U S A. 2008 Mar 11;105(10):3992-7. doi: 10.1073/pnas.0711700105. Epub 2008 Feb 28.


HIF-2alpha-haploinsufficient mice have blunted retinal neovascularization due to impaired expression of a proangiogenic gene battery.

Dioum EM, Clarke SL, Ding K, Repa JJ, Garcia JA.

Invest Ophthalmol Vis Sci. 2008 Jun;49(6):2714-20. doi: 10.1167/iovs.07-1469. Epub 2008 Feb 15.


Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse.

Repa JJ, Li H, Frank-Cannon TC, Valasek MA, Turley SD, Tansey MG, Dietschy JM.

J Neurosci. 2007 Dec 26;27(52):14470-80.


Genetic variations and treatments that affect the lifespan of the NPC1 mouse.

Liu B, Li H, Repa JJ, Turley SD, Dietschy JM.

J Lipid Res. 2008 Mar;49(3):663-9. Epub 2007 Dec 12.


Fenofibrate reduces intestinal cholesterol absorption via PPARalpha-dependent modulation of NPC1L1 expression in mouse.

Valasek MA, Clarke SL, Repa JJ.

J Lipid Res. 2007 Dec;48(12):2725-35. Epub 2007 Aug 28.


Regulation of antibacterial defense in the small intestine by the nuclear bile acid receptor.

Inagaki T, Moschetta A, Lee YK, Peng L, Zhao G, Downes M, Yu RT, Shelton JM, Richardson JA, Repa JJ, Mangelsdorf DJ, Kliewer SA.

Proc Natl Acad Sci U S A. 2006 Mar 7;103(10):3920-5. Epub 2006 Feb 10.


Fibroblast growth factor 15 functions as an enterohepatic signal to regulate bile acid homeostasis.

Inagaki T, Choi M, Moschetta A, Peng L, Cummins CL, McDonald JG, Luo G, Jones SA, Goodwin B, Richardson JA, Gerard RD, Repa JJ, Mangelsdorf DJ, Kliewer SA.

Cell Metab. 2005 Oct;2(4):217-25.


The power of real-time PCR.

Valasek MA, Repa JJ.

Adv Physiol Educ. 2005 Sep;29(3):151-9. Review.


Niemann-Pick C1 expression is not regulated by the amount of cholesterol flowing through cells in the mouse.

Garver WS, Xie C, Repa JJ, Turley SD, Dietschy JM.

J Lipid Res. 2005 Aug;46(8):1745-54. Epub 2005 Jun 1.


Caveolin-1 is not required for murine intestinal cholesterol transport.

Valasek MA, Weng J, Shaul PW, Anderson RG, Repa JJ.

J Biol Chem. 2005 Jul 29;280(30):28103-9. Epub 2005 May 26.


Differential regulation and properties of angiopoietin-like proteins 3 and 4.

Ge H, Cha JY, Gopal H, Harp C, Yu X, Repa JJ, Li C.

J Lipid Res. 2005 Jul;46(7):1484-90. Epub 2005 May 1.


Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease.

Li H, Repa JJ, Valasek MA, Beltroy EP, Turley SD, German DC, Dietschy JM.

J Neuropathol Exp Neurol. 2005 Apr;64(4):323-33.


Delineation of molecular changes in intrahepatic cholesterol metabolism resulting from diminished cholesterol absorption.

Repa JJ, Turley SD, Quan G, Dietschy JM.

J Lipid Res. 2005 Apr;46(4):779-89. Epub 2005 Jan 16.


Expression of ABCG5 and ABCG8 is required for regulation of biliary cholesterol secretion.

Yu L, Gupta S, Xu F, Liverman AD, Moschetta A, Mangelsdorf DJ, Repa JJ, Hobbs HH, Cohen JC.

J Biol Chem. 2005 Mar 11;280(10):8742-7. Epub 2004 Dec 16.


The nuclear xenobiotic receptor CAR: structural determinants of constitutive activation and heterodimerization.

Suino K, Peng L, Reynolds R, Li Y, Cha JY, Repa JJ, Kliewer SA, Xu HE.

Mol Cell. 2004 Dec 22;16(6):893-905.


ACAT2 deficiency limits cholesterol absorption in the cholesterol-fed mouse: impact on hepatic cholesterol homeostasis.

Repa JJ, Buhman KK, Farese RV Jr, Dietschy JM, Turley SD.

Hepatology. 2004 Nov;40(5):1088-97.


Quantitative real-time polymerase chain reaction measurement of regulators of G-protein signaling mRNA levels in mouse tissues.

Kurrasch DM, Huang J, Wilkie TM, Repa JJ.

Methods Enzymol. 2004;389:3-15. Review.


Regulation of the aldo-keto reductase gene akr1b7 by the nuclear oxysterol receptor LXRalpha (liver X receptor-alpha) in the mouse intestine: putative role of LXRs in lipid detoxification processes.

Volle DH, Repa JJ, Mazur A, Cummins CL, Val P, Henry-Berger J, Caira F, Veyssiere G, Mangelsdorf DJ, Lobaccaro JM.

Mol Endocrinol. 2004 Apr;18(4):888-98. Epub 2004 Jan 22.


Identification of a liver-specific uridine phosphorylase that is regulated by multiple lipid-sensing nuclear receptors.

Zhang Y, Repa JJ, Inoue Y, Hayhurst GP, Gonzalez FJ, Mangelsdorf DJ.

Mol Endocrinol. 2004 Apr;18(4):851-62. Epub 2004 Jan 8.


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