Research output per year
Research output per year
- 1 - 50 out of 99 results
Search results
-
2022
Molecular markers of brain cholesterol homeostasis are unchanged despite a smaller brain mass in a mouse model of cholesteryl ester storage disease
Aqul, A. A., Ramirez, C. M., Lopez, A. M., Burns, D. K., Repa, J. J. & Turley, S. D., Jan 2022, In: Lipids. 57, 1, p. 3-16 14 p.Research output: Contribution to journal › Article › peer-review
Open Access1 Scopus citations -
2021
Tonic prime-boost of STING signalling mediates Niemann–Pick disease type C
Chu, T. T., Tu, X., Yang, K., Wu, J., Repa, J. J. & Yan, N., Aug 26 2021, In: Nature. 596, 7873, p. 570-575 6 p.Research output: Contribution to journal › Article › peer-review
Open Access100 Scopus citations -
2020
Delineation of metabolic responses of Npc1−/−nih mice lacking the cholesterol-esterifying enzyme SOAT2 to acute treatment with 2-hydroxypropyl-β-cyclodextrin
Ramirez, C. M., Taylor, A. M., Lopez, A. M., Repa, J. J. & Turley, S. D., Dec 2020, In: Steroids. 164, 108725.Research output: Contribution to journal › Article › peer-review
Open Access4 Scopus citations -
Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann–Pick C1 Disease
Lopez, A. M., Ramirez, C. M., Taylor, A. M., Jones, R. D., Repa, J. J. & Turley, S. D., Jan 1 2020, In: Digestive Diseases and Sciences. 65, 1, p. 158-167 10 p.Research output: Contribution to journal › Article › peer-review
3 Scopus citations -
2019
1,25-Dihydroxyvitamin D 3 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, S. A., Vishwanath, M., Chuang, J. C., Posner, B. A., Mirmira, R. G. & Repa, J. J., Jan 2019, In: Journal of Steroid Biochemistry and Molecular Biology. 185, p. 17-26 10 p.Research output: Contribution to journal › Article › peer-review
Open Access34 Scopus citations -
2018
ACSS2 promotes systemic fat storage and utilization through selective regulation of genes involved in lipid metabolism
Huang, Z., Zhang, M., Plec, A. A., Estill, S. J., Cai, L., Repa, J. J., McKnight, S. L. & Tu, B. P., Oct 2 2018, In: Proceedings of the National Academy of Sciences of the United States of America. 115, 40, p. E9499-E9506Research output: Contribution to journal › Article › peer-review
Open Access85 Scopus citations -
Niemann-pick C1-deficient mice lacking sterol O-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function
Lopez, A. M., Jones, R. D., Repa, J. J. & Turley, S. D., Oct 2018, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 315, 4, p. G454-G463Research output: Contribution to journal › Article › peer-review
Open Access7 Scopus citations -
2017
Normalization of hepatic homeostasis in the Npc1nmf164 mouse model of niemann-pick type C disease treated with the histone deacetylase inhibitor vorinostat
Munkacsi, A. B., Hammond, N., Schneider, R. T., Senanayake, D. S., Higaki, K., Lagutin, K., Bloor, S. J., Ory, D. S., Maue, R. A., Chen, F. W., Hernandez-Ono, A., Dahlson, N., Repa, J. J., Ginsberg, H. N., Ioannou, Y. A. & Sturley, S. L., Mar 17 2017, In: Journal of Biological Chemistry. 292, 11, p. 4395-4410 16 p.Research output: Contribution to journal › Article › peer-review
Open Access24 Scopus citations -
2015
Impact of physiological levels of chenodeoxycholic acid supplementation on intestinal and hepatic bile acid and cholesterol metabolism in Cyp7a1-deficient mice
Jones, R. D., Lopez, A. M., Tong, E. Y., Posey, K. S., Chuang, J. C., Repa, J. J. & Turley, S. D., Jan 2015, In: Steroids. 93, p. 87-95 9 p.Research output: Contribution to journal › Article › peer-review
Open Access14 Scopus citations -
2014
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, K. V., Garbarino, J., Liu, Y., Moon, J., Schneider, K., Henneberry, A., Billheimer, J., Millar, J. S., Marchadier, D., Valasek, M. A., Joblin-Mills, A., Gulati, S., Munkacsi, A. B., Repa, J. J., Rader, D. & Sturley, S. L., Feb 14 2014, In: Journal of Biological Chemistry. 289, 7, p. 4417-4431 15 p.Research output: Contribution to journal › Article › peer-review
27 Scopus citations -
Hearing loss is an early consequence of Npc1 gene deletion in the mouse model of Niemann-Pick disease, type C
King, K. A., Gordon-Salant, S., Pawlowski, K. S., Taylor, A. M., Griffith, A. J., Houser, A., Kurima, K., Wassif, C. A., Wright, C. G., Porter, F. D., Repa, J. J. & Brewer, C. C., Aug 2014, In: JARO - Journal of the Association for Research in Otolaryngology. 15, 4, p. 529-541 13 p.Research output: Contribution to journal › Article › peer-review
Open Access15 Scopus citations -
Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient mice
Lopez, A. M., Posey, K. S., Repa, J. J., Turley, S. D., Aqul, A., Taylor, A. M., Repa, J. J., Burns, D. K. & Taylor, A. M., Oct 15 2014, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 307, 8, p. G836-G847Research output: Contribution to journal › Article › peer-review
Open Access23 Scopus citations -
Histone deacetylase 9 represses cholesterol efflux and alternatively activated macrophages in atherosclerosis development
Cao, Q., Rong, S., Repa, J. J., Clair, R. S., Parks, J. S. & Mishra, N., Sep 2014, In: Arteriosclerosis, thrombosis, and vascular biology. 34, 9, p. 1871-1879 9 p.Research output: Contribution to journal › Article › peer-review
Open Access140 Scopus citations -
Role of calcium and EPAC in norepinephrine-induced ghrelin secretion
Mani, B. K., Chuang, J. C., Kjalarsdottir, L., Sakata, I., Walker, A. K., Kuperman, A., Osborne-Lawrence, S., Repa, J. J. & Zigman, J. M., Jan 2014, In: Endocrinology. 155, 1, p. 98-107 10 p.Research output: Contribution to journal › Article › peer-review
20 Scopus citations -
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, J. C., Valasek, M. A., Lopez, A. M., Posey, K. S., Repa, J. J. & Turley, S. D., Apr 1 2014, In: Biochemical Pharmacology. 88, 3, p. 351-363 13 p.Research output: Contribution to journal › Article › peer-review
Open Access11 Scopus citations -
2013
Carboxylesterases are uniquely expressed among tissues and regulated by nuclear hormone receptors in the mouse
Jones, R. D., Taylor, A. M., Tong, E. Y. & Repa, J. J., Jan 2013, In: Drug Metabolism and Disposition. 41, 1, p. 40-49 10 p.Research output: Contribution to journal › Article › peer-review
Open Access67 Scopus citations -
Characterization of cholesterol homeostasis in telomeraseimmortalized 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., Dec 27 2013, In: Journal of Biological Chemistry. 288, 52, p. 36936-36947 12 p.Research output: Contribution to journal › Article › peer-review
10 Scopus citations -
The Xbp1s/GalE axis links ER stress to postprandial hepatic metabolism
Deng, Y., Wang, Z., Tao, C., Gao, N., Holland, W. L., Ferdous, A., Repa, J., Liang, G., Ye, J., Lehrman, M. A., Hill, J. A., Horton, J. D. & Scherer, P. E., Jan 2 2013, In: Journal of Clinical Investigation. 123, 1, p. 455-468 14 p.Research output: Contribution to journal › Article › peer-review
111 Scopus citations -
2012
Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream
Taylor, A. M., Liu, B., Mari, Y., Liu, B. & Repa, J. J., Nov 2012, In: Journal of lipid research. 53, 11, p. 2331-2342 12 p.Research output: Contribution to journal › Article › peer-review
58 Scopus citations -
Delineation of biochemical, molecular, and physiological changes accompanying bile acid pool size restoration in Cyp7a1 -/- mice fed low levels of cholic acid
Jones, R. D., Repa, J. J., Russell, D. W., Dietschy, J. M. & Turley, S. D., Jul 15 2012, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 303, 2, p. G263-G274Research output: Contribution to journal › Article › peer-review
Open Access18 Scopus citations -
2011
Ghrelin directly stimulates glucagon secretion from pancreatic α-cells
Chuang, J. C., Sakata, I., Kohno, D., Perello, M., Osborne-Lawrence, S., Repa, J. J. & Zigman, J. M., Sep 1 2011, In: Molecular Endocrinology. 25, 9, p. 1600-1611 12 p.Research output: Contribution to journal › Article › peer-review
Open Access100 Scopus citations -
Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations
Ramirez, C. M., Liu, B., Aqul, A., Taylor, A. M., Repa, J. J., Turley, S. D. & Dietschy, J. M., Apr 2011, In: Journal of lipid research. 52, 4, p. 688-698 11 p.Research output: Contribution to journal › Article › peer-review
59 Scopus citations -
Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment
Aqul, A., Liu, B., Ramirez, C. M., Pieper, A. A., Estill, S. J., Burns, D. K., Liu, B., Repa, J. J., Turley, S. D. & Dietschy, J. M., Jun 22 2011, In: Journal of Neuroscience. 31, 25, p. 9404-9413 10 p.Research output: Contribution to journal › Article › peer-review
125 Scopus citations -
2010
Chronic social defeat stress disrupts regulation of lipid synthesis
Chuang, J. C., Cui, H., Mason, B. L., Mahgoub, M., Bookout, A. L., Yu, H. G., Perello, M., Elmquist, J. K., Repa, J. J., Zigman, J. M. & Lutter, M., Jun 1 2010, In: Journal of lipid research. 51, 6, p. 1344-1353 10 p.Research output: Contribution to journal › Article › peer-review
93 Scopus citations -
Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid
Liu, B., Ramirez, C. M., Miller, A. M., Repa, J. J., Turley, S. D. & Dietschy, J. M., May 1 2010, In: Journal of lipid research. 51, 5, p. 933-944 12 p.Research output: Contribution to journal › Article › peer-review
142 Scopus citations -
Liver X receptor agonists augment human islet function through activation of anaplerotic pathways and glycerolipid/free fatty acid cycling
Ogihara, T., Chuang, J. C., Vestermark, G. L., Garmey, J. C., Ketchum, R. J., Huang, X., Brayman, K. L., Thorner, M. O., Repa, J. J., Mirmira, R. G. & Evans-Molina, C., Feb 19 2010, In: Journal of Biological Chemistry. 285, 8, p. 5392-5404 13 p.Research output: Contribution to journal › Article › peer-review
36 Scopus citations -
Multiple mechanisms limit the accumulation of unesterified cholesterol in the small intestine of mice deficient in both ACAT2 and ABCA1
Turley, S. D., Valasek, M. A., Repa, J. J. & Dietschy, J. M., Nov 2010, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 299, 5, p. G1012-G1022Research output: Contribution to journal › Article › peer-review
Open Access28 Scopus citations -
Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the niemann-pick type C1 mouse and markedly prolongs life
Ramirez, C. M., Liu, B., Taylor, A. M., Repa, J. J., Burns, D. K., Weinberg, A. G., Turley, S. D. & Dietschy, J. M., Oct 2010, In: Pediatric Research. 68, 4, p. 309-315 7 p.Research output: Contribution to journal › Article › peer-review
137 Scopus citations -
2009
Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse
Liu, B., Turley, S. D., Burns, D. K., Miller, A. M., Repa, J. J. & Dietschy, J. M., Feb 17 2009, In: Proceedings of the National Academy of Sciences of the United States of America. 106, 7, p. 2377-2382 6 p.Research output: Contribution to journal › Article › peer-review
Open Access331 Scopus citations -
2008
Elucidating a normal function of huntingtin by functional and microarray analysis of huntingtin-null mouse embryonic fibroblasts
Zhang, H., Das, S., Li, Q. Z., Dragatsis, I., Repa, J., Zeitlin, S., Hajnóczky, G. & Bezprozvanny, I., Apr 15 2008, In: BMC Neuroscience. 9, 38.Research output: Contribution to journal › Article › peer-review
25 Scopus citations -
Genetic variations and treatments that affect the lifespan of the NPC1 mouse
Liu, B., Li, H., Repa, J. J., Turley, S. D. & Dietschy, J. M., Mar 1 2008, In: Journal of lipid research. 49, 3, p. 663-669 7 p.Research output: Contribution to journal › Article › peer-review
135 Scopus citations -
GM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 mice
Li, H., Turley, S. D., Liu, B., Repa, J. J. & Dietschy, J. M., Aug 2008, In: Journal of lipid research. 49, 8, p. 1816-1828 13 p.Research output: Contribution to journal › Article › peer-review
24 Scopus citations -
HIF-2α-haploinsufficient mice have blunted retinal neovascularization due to impaired expression of a proangiogenic gene battery
Dioum, E. M., Clarke, S. L., Ding, K., Repa, J. J. & Garcia, J. A., Jun 2008, In: Investigative Ophthalmology and Visual Science. 49, 6, p. 2714-2720 7 p.Research output: Contribution to journal › Article › peer-review
33 Scopus citations -
Impaired insulin secretion and glucose intolerance in synaptotagmin-7 null mutant mice
Gustavsson, N., Lao, Y., Maximov, A., Chuang, J. C., Kostromina, E., Repa, J. J., Li, C., Radda, G. K., Südhof, T. C. & Han, W., Mar 25 2008, In: Proceedings of the National Academy of Sciences of the United States of America. 105, 10, p. 3992-3997 6 p.Research output: Contribution to journal › Article › peer-review
153 Scopus citations -
Inhibiting intestinal NPC1L1 activity prevents diet-induced increase in biliary cholesterol in Golden Syrian hamsters
Valasek, M. A., Repa, J. J., Quan, G., Dietschy, J. M. & Turley, S. D., Oct 2008, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 295, 4, p. G813-G822Research output: Contribution to journal › Article › peer-review
Open Access62 Scopus citations -
Nuclear hormone receptor expression in the endocrine pancreas
Chuang, J. C., Cha, J. Y., Garmey, J. C., Mirmira, R. G. & Repa, J. J., Oct 2008, In: Molecular Endocrinology. 22, 10, p. 2353-2363 11 p.Research output: Contribution to journal › Article › peer-review
55 Scopus citations -
Regulation of nuclear import/export of carbohydrate response element-binding protein (ChREBP): Interaction of anα-helix of ChREBP with the 14-3-3 proteins and regulation by phosphorylation
Sakiyama, H., Wynn, R. M., Lee, W. R., Fukasawa, M., Mizuguchi, H., Gardner, K. H., Repa, J. J. & Uyeda, K., Sep 5 2008, In: Journal of Biological Chemistry. 283, 36, p. 24899-24908 10 p.Research output: Contribution to journal › Article › peer-review
83 Scopus citations -
2007
Fenofibrate reduces intestinal cholesterol absorption via PPARα-dependent modulation of NPC1L1 expression in mouse
Valasek, M. A., Clarke, S. L. & Repa, J. J., Dec 2007, In: Journal of lipid research. 48, 12, p. 2725-2735 11 p.Research output: Contribution to journal › Article › peer-review
90 Scopus citations -
Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse
Repa, J. J., Li, H., Frank-Cannon, T. C., Valasek, M. A., Turley, S. D., Tansey, M. G. & Dietschy, J. M., Dec 26 2007, In: Journal of Neuroscience. 27, 52, p. 14470-14480 11 p.Research output: Contribution to journal › Article › peer-review
125 Scopus citations -
The Liver X Receptor (LXR) and hepatic lipogenesis: The carbohydrate-response element-binding protein is a target gene of LXR
Cha, J. Y. & Repa, J. J., Jan 5 2007, In: Journal of Biological Chemistry. 282, 1, p. 743-751 9 p.Research output: Contribution to journal › Article › peer-review
378 Scopus citations -
2006
Carbohydrate response element binding protein, ChREBP, a transcription factor coupling hepatic glucose utilization and lipid synthesis
Uyeda, K. & Repa, J. J., Aug 2006, In: Cell Metabolism. 4, 2, p. 107-110 4 p.Research output: Contribution to journal › Short survey › peer-review
421 Scopus citations -
Regulation of antibacterial defense in the small intestine by the nuclear bile acid receptor
Inagaki, T., Moschetta, A., Lee, Y. K., Peng, L., Zhao, G., Downes, M., Yu, R. T., Shelton, J. M., Richardson, J. A., Repa, J. J., Mangelsdorf, D. J. & Kliewer, S. A., Mar 7 2006, In: Proceedings of the National Academy of Sciences of the United States of America. 103, 10, p. 3920-3925 6 p.Research output: Contribution to journal › Article › peer-review
Open Access905 Scopus citations -
2005
Caveolin-1 is not required for murine intestinal cholesterol transport
Valasek, M. A., Weng, J., Shaul, P. W., Anderson, R. G. W. & Repa, J. J., Jul 29 2005, In: Journal of Biological Chemistry. 280, 30, p. 28103-28109 7 p.Research output: Contribution to journal › Article › peer-review
61 Scopus citations -
Delineation of molecular changes in intrahepatic cholesterol metabolism resulting from diminished cholesterol absorption
Repa, J. J., Turley, S. D., Quan, G. & Dietschy, J. M., 2005, In: Journal of lipid research. 46, 4, p. 779-789 11 p.Research output: Contribution to journal › Article › peer-review
85 Scopus citations -
Differential regulation and properties of angiopoietin-like proteins 3 and 4
Ge, H., Cha, J. Y., Gopal, H., Harp, C., Yu, X., Repa, J. J. & Li, C., 2005, In: Journal of lipid research. 46, 7, p. 1484-1490 7 p.Research output: Contribution to journal › Article › peer-review
126 Scopus citations -
Expression of ABCG5 and ABCG8 is required for regulation of biliary cholesterol secretion
Yu, L., Gupta, S., Xu, F., Liverman, A. D. B., Moschetta, A., Mangelsdorf, D. J., Repa, J. J., Hobbs, H. H. & Cohen, J. C., Mar 11 2005, In: Journal of Biological Chemistry. 280, 10, p. 8742-8747 6 p.Research output: Contribution to journal › Article › peer-review
192 Scopus citations -
Fibroblast growth factor 15 functions as an enterohepatic signal to regulate bile acid homeostasis
Inagaki, T., Choi, M., Moschetta, A., Peng, L., Cummins, C. L., McDonald, J. G., Luo, G., Jones, S. A., Goodwin, B., Richardson, J. A., Gerard, R. D., Repa, J. J., Mangelsdorf, D. J. & Kliewer, S. A., Oct 2005, In: Cell Metabolism. 2, 4, p. 217-225 9 p.Research output: Contribution to journal › Article › peer-review
1445 Scopus citations -
Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease
Li, H., Repa, J. J., Valasek, M. A., Beltroy, E. P., Turley, S. D., German, D. C. & Dietschy, J. M., Apr 2005, In: Journal of neuropathology and experimental neurology. 64, 4, p. 323-333 11 p.Research output: Contribution to journal › Article › peer-review
106 Scopus citations -
Niemann-Pick C1 expression is not regulated by the amount of cholesterol flowing through cells in the mouse
Garver, W. S., Xie, C., Repa, J. J., Turley, S. D. & Dietschy, J. M., Aug 2005, In: Journal of lipid research. 46, 8, p. 1745-1754 10 p.Research output: Contribution to journal › Article › peer-review
16 Scopus citations -
The power of real-time PCR
Valasek, M. A. & Repa, J. J., Sep 2005, In: American Journal of Physiology - Advances in Physiology Education. 29, 3, p. 151-159 9 p.Research output: Contribution to journal › Article › peer-review
438 Scopus citations