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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.


Characterization of cholesterol homeostasis in telomerase-immortalized Tangier disease fibroblasts reveals marked phenotype variability.

Kannenberg F, Gorzelniak K, Jäger K, Fobker M, Rust S, Repa J, Roth M, Björkhem I, Walter M.

J Biol Chem. 2013 Dec 27;288(52):36936-47. doi: 10.1074/jbc.M113.500256. Epub 2013 Nov 6.


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.

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