• 17980 Citations
  • 50 h-Index
1984 …2020

Research output per year

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Research Output

  • 17980 Citations
  • 50 h-Index
  • 90 Article
  • 6 Review article
  • 2 Short survey
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Article
2020

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 journalArticle

2018
4 Scopus citations

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-E9506

Research output: Contribution to journalArticle

8 Scopus citations
2 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 journalArticle

13 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 journalArticle

9 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 journalArticle

22 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 journalArticle

8 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-G847

Research output: Contribution to journalArticle

16 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 journalArticle

74 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 journalArticle

14 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 journalArticle

10 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 journalArticle

32 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 journalArticle

7 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 journalArticle

81 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 1 2012, In : Journal of lipid research. 53, 11, p. 2331-2342 12 p.

Research output: Contribution to journalArticle

43 Scopus citations
11 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 journalArticle

70 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 1 2011, In : Journal of lipid research. 52, 4, p. 688-698 11 p.

Research output: Contribution to journalArticle

43 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 journalArticle

97 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 journalArticle

67 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 journalArticle

112 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 journalArticle

33 Scopus citations
26 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 1 2010, In : Pediatric Research. 68, 4, p. 309-315 7 p.

Research output: Contribution to journalArticle

107 Scopus citations
2009
255 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 journalArticle

23 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 journalArticle

102 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 1 2008, In : Journal of lipid research. 49, 8, p. 1816-1828 13 p.

Research output: Contribution to journalArticle

21 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 1 2008, In : Investigative Ophthalmology and Visual Science. 49, 6, p. 2714-2720 7 p.

Research output: Contribution to journalArticle

29 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 journalArticle

125 Scopus citations
58 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 journalArticle

49 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 journalArticle

60 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 1 2007, In : Journal of lipid research. 48, 12, p. 2725-2735 11 p.

Research output: Contribution to journalArticle

76 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 journalArticle

117 Scopus citations
323 Scopus citations
2006

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 journalArticle

550 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 journalArticle

54 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 journalArticle

81 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 journalArticle

94 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 journalArticle

157 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 1 2005, In : Cell Metabolism. 2, 4, p. 217-225 9 p.

Research output: Contribution to journalArticle

1039 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 journalArticle

Open Access
95 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 1 2005, In : Journal of lipid research. 46, 8, p. 1745-1754 10 p.

Research output: Contribution to journalArticle

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 journalArticle

343 Scopus citations
2004

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

Repa, J. J., Buhman, K. K., Farese, R. V., Dietschy, J. M. & Turley, S. D., Nov 1 2004, In : Hepatology. 40, 5, p. 1088-1097 10 p.

Research output: Contribution to journalArticle

83 Scopus citations

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

Zhang, Y., Repa, J. J., Inoue, Y., Hayhurst, G. P., Gonzalez, F. J. & Mangelsdorf, D. J., Apr 1 2004, In : Molecular Endocrinology. 18, 4, p. 851-862 12 p.

Research output: Contribution to journalArticle

18 Scopus citations

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

Kurrasch, D. M., Huang, J., Wilkie, T. M. & Repa, J. J., Sep 3 2004, In : Methods in Enzymology. 389, p. 3-15 13 p.

Research output: Contribution to journalArticle

70 Scopus citations

Regulation of the aldo-keto reductase gene akr1b7 by the nuclear oxysterol receptor LXRα (liver X receptor-α) in the mouse intestine: Putative role of LXRs in lipid detoxification processes

Volle, D. H., Repa, J. J., Mazur, A., Cummins, C. L., Val, P., Henry-Berger, J., Caira, F., Veyssiere, G., Mangelsdorf, D. J. & Lobaccaro, J. M. A., Apr 2004, In : Molecular Endocrinology. 18, 4, p. 888-898 11 p.

Research output: Contribution to journalArticle

39 Scopus citations