Homozygous disruption of the murine MDR2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease

Multidrug resistance in cancer: role of ATP–dependent transporters

Gottesman, M. M., Fojo, T., & Bates, S. E. (2002) · Nature Reviews Cancer · doi:10.1038/nrc706 · Cited by 5,536

Lipopolysaccharide endotoxins

Raetz, C. R. H., & Whitfield, C. (2002) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.71.110601.135414 · Cited by 4,404

Targeted Disruption of the Nuclear Receptor FXR/BAR Impairs Bile Acid and Lipid Homeostasis

Sinal, C. J., Tohkin, M., Miyata, M., Ward, J. M., Lambert, G., & Gonzalez, F. J. (2000) · Cell · doi:10.1016/s0092-8674(00)00062-3 · Cited by 1,819

Lipids and Lipid-Based Formulations: Optimizing the Oral Delivery of Lipophilic Drugs

Porter, C. J. H., Trevaskis, N. L., & Charman, W. N. (2007) · Nature Reviews Drug Discovery · doi:10.1038/nrd2197 · Cited by 1,824

Role of Bile Acids and Bile Acid Receptors in Metabolic Regulation

Lefebvre, P., Cariou, B., Lien, F., Kuipers, F., & Staels, B. (2009) · Physiological Reviews · doi:10.1152/physrev.00010.2008 · Cited by 1,678

A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Starqardt macular dystrophy

Allikmets, R., Singh, N., Sun, H., Shroyer, N. F., Hutchinson, A., Chidambaram, A., Gerrard, B., Baird, L., Stauffer, D., Peiffer, A., et al. (1997) · Nature Genetics · doi:10.1038/ng0397-236 · Cited by 1,435

Pathophysiologic Implications of Membrane Phospholipid Asymmetry in Blood Cells

Zwaal, R. F. A., & Schroit, A. J. (1997) · Blood · doi:10.1182/blood.v89.4.1121 · Cited by 1,340

Bile salt transporters: molecular characterization, function, and regulation

Trauner, M., & Boyer, J. L. (2003) · Physiological Reviews · doi:10.1152/physrev.00027.2002 · Cited by 999

Bile acid signaling in metabolic disease and drug therapy

Li, T., & Chiang, J. Y. L. (2014) · Pharmacological Reviews · doi:10.1124/pr.113.008201 · Cited by 935

Insights into the Function of Rim Protein in Photoreceptors and Etiology of Stargardt's Disease from the Phenotype in abcr Knockout Mice

Weng, J., Mata, N. L., Azarian, S. M., Tzekov, R. T., Birch, D. G., & Travis, G. H. (1999) · Cell · doi:10.1016/s0092-8674(00)80602-9 · Cited by 881

Primary sclerosing cholangitis – a comprehensive review

Karlsen, T. H., Folseraas, T., Thorburn, D., & Vesterhus, M. (2017) · Journal of Hepatology · doi:10.1016/j.jhep.2017.07.022 · Cited by 868

The Tangier disease gene product ABC1 controls the cellular apolipoprotein-mediated lipid removal pathway

Lawn, R. M., Wade, D. P., Garvin, M. R., Wang, X., Schwartz, K., Porter, J. G., Seilhamer, J. J., Vaughan, A. M., & Oram, J. F. (1999) · Journal of Clinical Investigation · doi:10.1172/jci8119 · Cited by 724

Cholesterol gallstone disease

Portincasa, P., Moschetta, A., & Palasciano, G. (2006) · The Lancet · doi:10.1016/s0140-6736(06)69044-2 · Cited by 643

ATP-Binding Cassette Cholesterol Transporters and Cardiovascular Disease

Oram, J. F., & Vaughan, A. M. (2006) · Circulation Research · doi:10.1161/01.res.0000250171.54048.5c · Cited by 411

Confronting Complexity: the Interlink of Phototransduction and Retinoid Metabolism in the Vertebrate Retina

McBee, J. K., Palczewski, K., Baehr, W., & Pepperberg, D. R. (2001) · Progress in Retinal and Eye Research · doi:10.1016/s1350-9462(01)00002-7 · Cited by 366

A Mouse Model of Hepatocellular Carcinoma

Nicholes, K., Guillet, S., Tomlinson, E., Hillan, K., Wright, B., Frantz, G. D., Pham, T. A., Dillard-Telm, L., Tsai, S. P., Stephan, J.-P., et al. (2002) · The American Journal of Pathology · doi:10.1016/s0002-9440(10)61177-7 · Cited by 359

Fibrates and cholestasis

Ghonem, N. S., Assis, D. N., & Boyer, J. L. (2015) · Hepatology · doi:10.1002/hep.27744 · Cited by 317

Selective activation of nuclear bile acid receptor FXR in the intestine protects mice against cholestasis

Modica, S., Petruzzelli, M., Bellafante, E., Murzilli, S., Salvatore, L., Celli, N., Di Tullio, G., Palasciano, G., Moustafa, T., Halilbasic, E., et al. (2012) · Gastroenterology · doi:10.1053/j.gastro.2011.10.028 · Cited by 275

Absence of the intestinal microbiota exacerbates hepatobiliary disease in a murine model of primary sclerosing cholangitis

Tabibian, J. H., O'Hara, S. P., Trussoni, C. E., Tietz, P. S., Splinter, P. L., Mounajjed, T., Hagey, L. R., & LaRusso, N. F. (2016) · Hepatology · doi:10.1002/hep.27927 · Cited by 223

Correction of liver disease by hepatocyte transplantation in a mouse model of progressive familial intrahepatic cholestasis

De Vree, J. M. L., Ottenhoff, R., Bosma, P. J., Smith, A. J., Aten, J., & Oude Elferink, R. P. J. (2000) · Gastroenterology · doi:10.1053/gast.2000.20222 · Cited by 186

Nuclear receptors in bile acid metabolism

Li, T., & Chiang, J. Y. L. (2013) · Drug Metabolism Reviews · doi:10.3109/03602532.2012.740048 · Cited by 176

Recurrence and rejection in liver transplantation for primary sclerosing cholangitis

Fosby, B. (2012) · World Journal of Gastroenterology · doi:10.3748/wjg.v18.i1.1 · Cited by 175

Lineage fate of ductular reactions in liver injury and carcinogenesis

Jörs, S., Jeliazkova, P., Ringelhan, M., Thalhammer, J., Dürl, S., Ferrer, J., Sander, M., Heikenwalder, M., Schmid, R. M., Siveke, J. T., et al. (2015) · Journal of Clinical Investigation · doi:10.1172/jci78585 · Cited by 149

A “forward genomics” approach links genotype to phenotype using independent phenotypic losses among related species

Hiller, M., Schaar, B. T., Indjeian, V. B., Kingsley, D. M., Hagey, L. R., & Bejerano, G. (2012) · Cell Reports · doi:10.1016/j.celrep.2012.08.032 · Cited by 144

Alterations in Intestinal Microbiota Lead to Production of Interleukin 17 by Intrahepatic γδ T-Cell Receptor–Positive Cells and Pathogenesis of Cholestatic Liver Disease

Tedesco, D., Thapa, M., Chin, C. Y., Ge, Y., Gong, M., Li, J., Gumber, S., Speck, P., Elrod, E. J., Burd, E. M., et al. (2018) · Gastroenterology · doi:10.1053/j.gastro.2018.02.019 · Cited by 137

Bezafibrate treatment: a new medical approach for PBC patients?

Kanda, T., Yokosuka, O., Imazeki, F., & Saisho, H. (2003) · Journal of Gastroenterology · doi:10.1007/s00535-002-1102-7 · Cited by 129

Bile Formation and Secretion

Boyer, J. L. (2013) · Comprehensive Physiology · doi:10.1002/j.2040-4603.2013.tb00519.x · Cited by 124

Mouse species-specific control of hepatocarcinogenesis and metabolism by FGF19/FGF15

Zhou, M., Luo, J., Chen, M., Yang, H., Learned, R. M., DePaoli, A. M., Tian, H., & Ling, L. (2017) · Journal of Hepatology · doi:10.1016/j.jhep.2017.01.027 · Cited by 102

ABCB4 gene aberrations in human liver disease: an evolving spectrum

Reichert, M., & Lammert, F. (2018) · Seminars in Liver Disease · doi:10.1055/s-0038-1667299 · Cited by 81

Na + -taurocholate cotransporting polypeptide inhibition has hepatoprotective effects in cholestasis in mice

Slijepcevic, D., Roscam Abbing, R. L. P., Fuchs, C. D., Haazen, L. C. M., Beuers, U., Trauner, M., Oude Elferink, R. P. J., & van de Graaf, S. F. J. (2018) · Hepatology · doi:10.1002/hep.29888 · Cited by 73

Combined hepatocellular-cholangiocarcinoma derives from liver progenitor cells and depends on senescence and IL-6 trans-signaling

Rosenberg, N., Van Haele, M., Lanton, T., Brashi, N., Bromberg, Z., Adler, H., Giladi, H., Peled, A., Goldenberg, D. S., Axelrod, J. H., et al. (2022) · Journal of Hepatology · doi:10.1016/j.jhep.2022.07.029 · Cited by 72

Will we ever model PSC? – “It's hard to be a PSC model!”

Pollheimer, M. J., Trauner, M., & Fickert, P. (2011) · Clinics and Research in Hepatology and Gastroenterology · doi:10.1016/j.clinre.2011.04.014 · Cited by 66

The Origin and Fate of Liver Myofibroblasts

Kim, H. Y., Sakane, S., Eguileor, A., Carvalho Gontijo Weber, R., Lee, W., Liu, X., Lam, K., Ishizuka, K., Rosenthal, S. B., Diggle, K., et al. (2024) · Cellular and Molecular Gastroenterology and Hepatology · doi:10.1016/j.jcmgh.2023.09.008 · Cited by 63

The ABCs of Sterol Transport

Plummer, A. M., Culbertson, A. T., & Liao, M. (2021) · Annual Review of Physiology · doi:10.1146/annurev-physiol-031620-094944 · Cited by 60

Bezafibrate stimulates canalicular localization of NBD-labeled PC in HepG2 cells by PPARα-mediated redistribution of ABCB4

Shoda, J., Inada, Y., Tsuji, A., Kusama, H., Ueda, T., Ikegami, T., Suzuki, H., Sugiyama, Y., Cohen, D. E., & Tanaka, N. (2004) · Journal of Lipid Research · doi:10.1194/jlr.m400132-jlr200 · Cited by 58

The liver as a central "hub" of the immune system: pathophysiological implications

Ronca, V., Gerussi, A., Collins, P., Parente, A., Oo, Y. H., & Invernizzi, P. (2025) · Physiological Reviews · doi:10.1152/physrev.00004.2023 · Cited by 59

Intestinal mucosal damage caused by non-steroidal anti-inflammatory drugs: role of bile salts

Petruzzelli, M., Vacca, M., Moschetta, A., Cinzia Sasso, R., Palasciano, G., van Erpecum, K. J., & Portincasa, P. (2007) · Clinical Biochemistry · doi:10.1016/j.clinbiochem.2007.01.015 · Cited by 46

The Role of B Cells and B Cell Therapies in Immune-Mediated Liver Diseases

Cargill, T., & Culver, E. L. (2021) · Frontiers in Immunology · doi:10.3389/fimmu.2021.661196 · Cited by 46

Disturbed gut microbiota and bile homeostasis in Giardia -infected mice contributes to metabolic dysregulation and growth impairment

Riba, A., Hassani, K., Walker, A., van Best, N., von Zeschwitz, D., Anslinger, T., Sillner, N., Rosenhain, S., Eibach, D., Maiga-Ascofaré, O., et al. (2020) · Science Translational Medicine · doi:10.1126/scitranslmed.aay7019 · Cited by 45

Mouse liver assembloids model periportal architecture and biliary fibrosis

Dowbaj, A. M., Sljukic, A., Niksic, A., Landerer, C., Delpierre, J., Yang, H., Lahree, A., Kühn, A. C., Beers, D., Byrne, H. M., et al. (2025) · Nature · doi:10.1038/s41586-025-09183-9 · Cited by 40

Lysyl oxidase-like protein 2 (LOXL2) modulates barrier function in cholangiocytes in cholestasis

Pollheimer, M. J., Racedo, S., Mikels-Vigdal, A., Marshall, D., Bowlus, C., Lackner, C., Madl, T., Karlsen, T. H., Hov, J. R., Lyman, S. K., et al. (2018) · Journal of Hepatology · doi:10.1016/j.jhep.2018.04.009 · Cited by 38

All-Trans-Retinoic Acid Improves Cholestasis in α-Naphthylisothiocyanate–Treated Rats and Mdr2−/− Mice

Cai, S.-Y., Mennone, A., Soroka, C. J., & Boyer, J. L. (2014) · The Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.113.209353 · Cited by 36

Lack of enteral nutrition during critical illness is associated with profound decrements in biliary lipid concentrationsrted by the Dutch Organization for Scientific Research, project no. 902-23-097.rted by the Dutch Organization for Scientific Research, project no. 902-23-097.rted by the Dutch Organization for Scientific Research, project no. 902-23-097.

de Vree, J. M. L., Romijn, J. A., Mok, K. S., Mathus-Vliegen, L. M., Stoutenbeek, C. P., Ostrow, J. D., Tytgat, G. N., Sauerwein, H. P., Elferink, R. P. O., & Groen, A. K. (1999) · The American Journal of Clinical Nutrition · doi:10.1093/ajcn/70.1.70 · Cited by 35

Effects of pemafibrate on primary biliary cholangitis with dyslipidemia

Yamaguchi, M., Asano, T., Arisaka, T., Mashima, H., Irisawa, A., & Tamano, M. (2022) · Hepatology Research · doi:10.1111/hepr.13747 · Cited by 23

Blockade of BAFF Reshapes the Hepatic B Cell Receptor Repertoire and Attenuates Autoantibody Production in Cholestatic Liver Disease

Thapa, M., Tedesco, D., Gumber, S., Elrod, E. J., Han, J.-H., Kitchens, W. H., Magliocca, J. F., Adams, A. B., & Grakoui, A. (2020) · The Journal of Immunology · doi:10.4049/jimmunol.1900391 · Cited by 20

Effects of Switching from Fenofibrate to Pemafibrate for Asymptomatic Primary Biliary Cholangitis

Dohmen, K., Onohara, S.-Y., & Harada, S. (2021) · The Korean Journal of Gastroenterology · doi:10.4166/kjg.2021.092 · Cited by 18