Pathogenesis of Cholestatic Liver Disease and Therapeutic Approaches

Immunol. Cell Biol.

Fitzgerald, M., Hayward, I. P., Thomas, A. C., Campbell, J. H., & CAMPBELL, G. R. (1995) · Cited by 2,552

SLCO1B1 Variants and Statin-Induced Myopathy — A Genomewide Study

(2008) · New England Journal of Medicine · doi:10.1056/nejmoa0801936 · Cited by 2,043

Primary Biliary Cirrhosis

Kaplan, M. M. (1987) · New England Journal of Medicine · doi:10.1056/nejm198702263160907 · Cited by 2,016

EASL Clinical Practice Guidelines: management of cholestatic liver diseases

European Association for the Study of the Liver (2009) · Journal of Hepatology · doi:10.1016/j.jhep.2009.04.009 · Cited by 1,729

Interleukin-17 and Type 17 Helper T Cells

Miossec, P., Korn, T., & Kuchroo, V. K. (2009) · New England Journal of Medicine · doi:10.1056/nejmra0707449 · Cited by 1,409

Primary Biliary Cirrhosis

Schwab, M. (2011) · Springer eBooks · doi:10.1007/978-3-642-16483-5_4730 · Cited by 1,241

Diagnosis and Management of Primary Sclerosing Cholangitis

Chapman, R. W., Fevery, J., Kalloo, A. N., Nagorney, D. M., Boberg, K. M., Shneider, B. L., & Gores, G. J. (2009) · Hepatology · doi:10.1002/hep.23294 · Cited by 1,230

Primary biliary cirrhosis

Kaplan, M. M., & Gershwin, M. E. (2005) · New England Journal of Medicine · doi:10.1056/nejmra043898 · Cited by 1,144

Primary biliary cirrhosis # †

Lindor, K. D., Gershwin, E. M., Poupon, R., Kaplan, M., Bergasa, N. V., & Heathcote, J. E. (2009) · Hepatology · doi:10.1002/hep.22906 · Cited by 1,099

HLA-B*5701 genotype is a major determinant of drug-induced liver injury due to flucloxacillin

study, F. T. D., Daly, A. K., Donaldson, P. T., Bhatnagar, P., Shen, Y., Pe’er, I., Floratos, A., Daly, M. J., Goldstein, D. B., John, S., et al. (2009) · Nature Genetics · doi:10.1038/ng.379 · Cited by 1,050

Th17 and regulatory T cells in mediating and restraining inflammation

Littman, D. R., & Rudensky, A. Y. (2010) · Cell · doi:10.1016/j.cell.2010.02.021 · Cited by 1,039

Generation of Gut-Homing IgA-Secreting B Cells by Intestinal Dendritic Cells

Mora, J. R., Iwata, M., Eksteen, B., Song, S., Junt, T., Senman, B. C., Otipoby, K. L., Yokota, A., Takeuchi, H., Ricciardi‐Castagnoli, P., et al. (2006) · Science · doi:10.1126/science.1132742 · Cited by 1,014

Primary Biliary Cirrhosis

(2015) · Elsevier eBooks · doi:10.1016/b978-0-323-37755-3.50202-6 · Cited by 808

PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis

Loftus, E. V., Harewood, G. C., Loftus, C. G., Tremaine, W. J., Harmsen, W. S., Zinsmeister, A. R., Jewell, D. A., & Sandborn, W. J. (2005) · Gut · doi:10.1136/gut.2004.046615 · Cited by 777

Excellent Long-Term Survival in Patients With Primary Biliary Cirrhosis and Biochemical Response to Ursodeoxycholic Acid

Parés, A., Caballería, L., & Rodés, J. (2006) · Gastroenterology · doi:10.1053/j.gastro.2005.12.029 · Cited by 756

A Multicenter, Controlled Trial of Ursodiol for the Treatment of Primary Biliary Cirrhosis

Poupon, R. E., Balkau, B., Eschwège, E., Poupon, R., & the UDCA–PBC Study Group* (1991) · New England Journal of Medicine · doi:10.1056/nejm199105303242204 · Cited by 755

High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis

Lindor, K. D., Kowdley, K. V., Luketic, V. A. C., Harrison, E. M., McCashland, T., Befeler, A. S., Harnois, D., Jorgensen, R., Petz, J., Keach, J., et al. (2009) · Hepatology · doi:10.1002/hep.23082 · Cited by 720

Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis

Poupon, R., Lindor, K., Cauch-Dudek, K., Dickson, E., Poupon, R., & Heathcote, E. (1997) · Gastroenterology · doi:10.1016/s0016-5085(97)70183-5 · Cited by 648

Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis

Corpechot, C., Abenavoli, L., Rabahi, N., Chrétien, Y., Andréani, T., Johanet, C., Chazouillères, O., & Poupon, R. (2008) · Hepatology · doi:10.1002/hep.22428 · Cited by 646

Farnesoid X receptor antagonizes nuclear factor κB in hepatic inflammatory response

Wang, Y.-D., Chen, W.-D., Wang, M., Yu, D., Forman, B. M., & Huang, W. (2008) · Hepatology · doi:10.1002/hep.22519 · Cited by 639

Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview‐based study of 1032 patients

Gershwin, E. M., Selmi, C., Worman, H. J., Gold, E. B., Watnik, M., Utts, J., Lindor, K. D., Kaplan, M. M., & Vierling, J. M. (2005) · Hepatology · doi:10.1002/hep.20907 · Cited by 621

Primary Biliary Cirrhosis Associated with HLA, IL12A, and IL12RB2 Variants

Hirschfield, G. M., Liu, X., Xu, C., Lu, Y., Xie, G., Lu, Y., Gu, X., Walker, E. J., Jing, K., Juran, B. D., et al. (2009) · New England Journal of Medicine · doi:10.1056/nejmoa0810440 · Cited by 594

Identification and specificity of a cDNA encoding the 70 kd mitochondrial antigen recognized in primary biliary cirrhosis

Gershwin, M. E., Mackay, I. R., Sturgess, A., & Coppel, R. L. (1987) · The Journal of Immunology · doi:10.4049/jimmunol.138.10.3525 · Cited by 557

ARE STEROID INHALERS SAFER THAN TABLETS?

Brown, H. M., Jackson, F., & Herxheimer, H. (1979) · The Lancet · doi:10.1016/s0140-6736(79)91347-3 · Cited by 518

SEROLOGICAL TESTS IN DIAGNOSIS OF PRIMARY BILIARY CIRRHOSIS

WALKER, J. (1965) · The Lancet · doi:10.1016/s0140-6736(65)91372-3 · Cited by 507

Ursodiol for the Long-Term Treatment of Primary Biliary Cirrhosis

Poupon, R. E., Poupon, R., & Balkau, B. (1994) · New England Journal of Medicine · doi:10.1056/nejm199405123301903 · Cited by 497

Primary biliary cirrhosis in monozygotic and dizygotic twins: Genetics, epigenetics, and environment

Selmi, C., Mayo, M. J., Bach, N., Ishibashi, H., Invernizzi, P., Gish, R. G., Gordon, S. C., Wright, H. I., Zweiban, B., Podda, M., et al. (2004) · Gastroenterology · doi:10.1053/j.gastro.2004.05.005 · Cited by 480

The nuclear receptor SHP mediates inhibition of hepatic stellate cells by FXR and protects against liver fibrosis

Fiorucci, S., Antonelli, E., Rizzo, G., Renga, B., Mencarelli, A., Riccardi, L., Orlandi, S., Pellicciari, R., & Morelli, A. (2004) · Gastroenterology · doi:10.1053/j.gastro.2004.08.001 · Cited by 472

Regurgitation of bile acids from leaky bile ducts causes sclerosing cholangitis in Mdr2 (Abcb4) knockout mice

Fickert, P., Fuchsbichler, A., Wagner, M., Zollner, G., Kaser, A., Tilg, H., Krause, R. G. E., Lammert, F., Langner, C., Zatloukal, K., et al. (2004) · Gastroenterology · doi:10.1053/j.gastro.2004.04.009 · Cited by 468

Improved Prognosis of Patients With Primary Biliary Cirrhosis That Have a Biochemical Response to Ursodeoxycholic Acid

Kuiper, E. M. M., Hansen, B. E., de Vries, R. A., den Ouden–Muller, J. W., van Ditzhuijsen, T. J. M., Haagsma, E. B., Houben, M. H. M. G., Witteman, B. J. M., van Erpecum, K. J., & van Buuren, H. R. (2009) · Gastroenterology · doi:10.1053/j.gastro.2009.01.003 · Cited by 460

Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: A tight junction disease

Hadj-Rabia, S., Baala, L., Vabres, P., Hamel‐Teillac, D., Jacquemin, E., Fabrè, M., Lyonnet, S., Prost, Y. D., Münnich, A., Hadchouel, M., et al. (2004) · Gastroenterology · doi:10.1053/j.gastro.2004.07.022 · Cited by 426

Drug Insight: mechanisms and sites of action of ursodeoxycholic acid in cholestasis

Beuers, U. (2006) · Nature Clinical Practice Gastroenterology & Hepatology · doi:10.1038/ncpgasthep0521 · Cited by 417

High lifetime risk of cancer in primary sclerosing cholangitis

Claessen, M. M., Vleggaar, F. P., Tytgat, K. M. A. J., Siersema, P. D., & van Buuren, H. R. (2008) · Journal of Hepatology · doi:10.1016/j.jhep.2008.08.013 · Cited by 408

The apical conjugate efflux pump ABCC2 (MRP2)

Nies, A. T., & KEPPLER, D. O. R. (2006) · Pflügers Archiv - European Journal of Physiology · doi:10.1007/s00424-006-0109-y · Cited by 401

Genome-wide meta-analyses identify three loci associated with primary biliary cirrhosis

Liu, X., Invernizzi, P., Lu, Y., Kosoy, R., Lu, Y., Bianchi, I., Podda, M., Xu, C., Xie, G., Macciardi, F., et al. (2010) · Nature Genetics · doi:10.1038/ng.627 · Cited by 398

Baseline Ductopenia and Treatment Response Predict Long-Term Histological Progression in Primary Biliary Cirrhosis

Kumagi, T., Guindi, M., Fischer, S. E., Arenovich, T., Abdalian, R., Coltescu, C., Heathcote, J. E., & Hirschfield, G. M. (2010) · American Journal of Gastroenterology · doi:10.1038/ajg.2010.216 · Cited by 372

Hepatic Endothelial CCL25 Mediates the Recruitment of CCR9+ Gut-homing Lymphocytes to the Liver in Primary Sclerosing Cholangitis

Eksteen, B., Grant, A. J., Miles, A., Curbishley, S. M., Lalor, P. F., Hübscher, S. G., Briskin, M., Salmon, M., & Adams, D. (2004) · The Journal of Experimental Medicine · doi:10.1084/jem.20041035 · Cited by 366

Genome-Wide Association Analysis in Primary Sclerosing Cholangitis

Karlsen, T. H., Franke, A., Melum, E., Kaser, A., Hov, J. R., Balschun, T. C., Lie, B. A., Bergquist, A. M., Schramm, C., Weismüller, T. J., et al. (2009) · Gastroenterology · doi:10.1053/j.gastro.2009.11.046 · Cited by 365

Pharmacogenomics of human OATP transporters

König, J., Seithel‐Keuth, A., Gradhand, U., & Fromm, M. F. (2006) · Naunyn-Schmiedeberg s Archives of Pharmacology · doi:10.1007/s00210-006-0040-y · Cited by 358

Identification of a Novel Antibody Associated with Autoimmune Pancreatitis

Frulloni, L., Lunardi, C., Simone, R., Dolcino, M., Scattolini, C., Falconi, M., Benini, L., Vantini, I., Corrocher, R., & Puccetti, A. (2009) · New England Journal of Medicine · doi:10.1056/nejmoa0903068 · Cited by 355

The Biliary HCO3 − Umbrella: A Unifying Hypothesis on Pathogenetic and Therapeutic Aspects of Fibrosing Cholangiopathies

Beuers, U., Hohenester, S., de Buy Wenniger, L. J. M., Kremer, A. E., Jansen, P. L. M., & Elferink, R. P. J. O. (2010) · Hepatology · doi:10.1002/hep.23810 · Cited by 351

Hedgehog signaling regulates epithelial-mesenchymal transition during biliary fibrosis in rodents and humans

Omenetti, A., Porrello, A., Jung, Y., Yang, L., Popov, Y. V., Choi, S. S., Witek, R. P., Alpini, G. D., Venter, J., VanDongen, H. M. A., et al. (2008) · Journal of Clinical Investigation · doi:10.1172/jci35875 · Cited by 346

Mutations in VPS33B, encoding a regulator of SNARE-dependent membrane fusion, cause arthrogryposis–renal dysfunction–cholestasis (ARC) syndrome

Gissen, P., Johnson, C. A., Morgan, N. V., Stapelbroek, J. M., Forshew, T., Cooper, W. N., McKiernan, P. J., Klomp, L. W. J., Morris, A. A. M., Wraith, J. E., et al. (2004) · Nature Genetics · doi:10.1038/ng1325 · Cited by 341

Epithelial‐to‐mesenchymal transitions in the liver†

Choi, S. S., & Diehl, A. M. (2009) · Hepatology · doi:10.1002/hep.23196 · Cited by 314

Patients With Primary Biliary Cirrhosis React Against A Ubiquitous Xenobiotic–Metabolizing Bacterium

Selmi, C., Balkwill, D. L., Invernizzi, P., Ansari, A. A., Coppel, R. L., Podda, M., Leung, P. S., Kenny, T. P., Van De Water, J., Nantz, M. H., et al. (2003) · Hepatology · doi:10.1053/jhep.2003.50446 · Cited by 312

Liver‐targeted and peripheral blood alterations of regulatory T cells in primary biliary cirrhosis†

Lan, R. Y., Cheng, C., Lian, Z., Tsuneyama, K., Yang, G., Moritoki, Y., Chuang, Y., Nakamura, T., Saito, S., Shimoda, S., et al. (2006) · Hepatology · doi:10.1002/hep.21123 · Cited by 304

HLA DRB4 0101-restricted immunodominant T cell autoepitope of pyruvate dehydrogenase complex in primary biliary cirrhosis: evidence of molecular mimicry in human autoimmune diseases.

Shimoda, S., Nakamura, M., Ishibashi, H., Hayashida, K., & Niho, Y. (1995) · The Journal of Experimental Medicine · doi:10.1084/jem.181.5.1835 · Cited by 304

24-norUrsodeoxycholic Acid Is Superior to Ursodeoxycholic Acid in the Treatment of Sclerosing Cholangitis in Mdr2 (Abcb4) Knockout Mice

Fickert, P., Wagner, M., Marschall, H., Fuchsbichler, A., Zollner, G., Tsybrovskyy, O., Zatloukal, K., Liu, J., Waalkes, M. P., Cover, C., et al. (2006) · Gastroenterology · doi:10.1053/j.gastro.2005.10.018 · Cited by 301

Cholangiocyte Cilia Detect Changes in Luminal Fluid Flow and Transmit Them Into Intracellular Ca2+ and cAMP Signaling

Masyuk, A. I., Masyuk, T. V., Splinter, P. L., Huang, B. Q., Stroope, A. J., & LaRusso, N. F. (2006) · Gastroenterology · doi:10.1053/j.gastro.2006.07.003 · Cited by 293

The pathobiology of biliary epithelia

Alpini, G., McGill, J. M., & LaRusso, N. (2002) · Hepatology · doi:10.1053/jhep.2002.33541 · Cited by 293

PRIMARY BILIARY CIRRHOSIS: IDENTIFICATION OF TWO MAJOR M2 MITOCHONDRIAL AUTOANTIGENS

Yeaman, S. J., Danner, D. J., Mutimer, D., Fussey, S. P. M., James, O., & Bassendine, M. F. (1988) · The Lancet · doi:10.1016/s0140-6736(88)91894-6 · Cited by 292

Identification of HLA-A2–restricted CD8+ Cytotoxic T Cell Responses in Primary Biliary Cirrhosis

Kita, H., Lian, Z., de Water, J. V., He, X.-S., Matsumura, S., Kaplan, M., Luketic, V. A., Coppel, R. L., Ansari, A. A., & Gershwin, M. E. (2002) · The Journal of Experimental Medicine · doi:10.1084/jem.20010956 · Cited by 292

The membrane-bound bile acid receptor TGR5 is localized in the epithelium of human gallbladders #

Keitel, V., Cupisti, K., Ullmer, C., Knoefel, W. T., Kubitz, R., & Häussinger, D. (2009) · Hepatology · doi:10.1002/hep.23032 · Cited by 290

Primary biliary cirrhosis: an orchestrated immune response against epithelial cells

Gershwin, M. E., Ansari, A. A., Mackay, I. R., Nakanuma, Y., Nishio, A., Rowley, M. J., & Coppel, R. L. (2000) · Immunological Reviews · doi:10.1034/j.1600-0528.2002.017402.x · Cited by 275

Identification and analysis of the major M2 autoantigens in primary biliary cirrhosis.

Fussey, S. P., Guest, J. R., James, O. F., Bassendine, M. F., & Yeaman, S. J. (1988) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.85.22.8654 · Cited by 273

Frequency of monosomy X in women with primary biliary cirrhosis

Invernizzi, P., Miozzo, M. R., Battezzati, P. M., Bianchi, I., Grati, F. R., Simoni, G., Selmi, C. F., Watnik, M., Gershwin, M. E., & Podda, M. (2004) · The Lancet · doi:10.1016/s0140-6736(04)15541-4 · Cited by 268

Homing of mucosal lymphocytes to the liver in the pathogenesis of hepatic complications of inflammatory bowel disease

Grant, A. J., Lalor, P. F., Salmi, M., Jalkanen, S., & Adams, D. (2002) · The Lancet · doi:10.1016/s0140-6736(02)07374-9 · Cited by 255

Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase.

Coppel, R. L., McNeilage, L. J., Surh, C. D., de Water, J. V., Spithill, T. W., Whittingham, S., & Gershwin, M. E. (1988) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.85.19.7317 · Cited by 252

Identification and precursor frequency analysis of a common T cell epitope motif in mitochondrial autoantigens in primary biliary cirrhosis.

Shimoda, S., de Water, J. V., Ansari, A. A., Nakamura, M., Ishibashi, H., Coppel, R. L., Lake, J. R., Keeffe, E. B., Roche, T. E., & Gershwin, M. E. (1998) · Journal of Clinical Investigation · doi:10.1172/jci4213 · Cited by 250

Anti-Mitochondrial Antibodies and Primary Biliary Cirrhosis in TGF-β Receptor II Dominant-Negative Mice

Oertelt‐Prigione, S., Lian, Z., Cheng, C.-M., Chuang, Y., Padgett, K. A., He, X.-S., Ridgway, W. M., Ansari, A. A., Coppel, R. L., Li, M. O., et al. (2006) · The Journal of Immunology · doi:10.4049/jimmunol.177.3.1655 · Cited by 248

The Spectrum of Liver Diseases Related toABCB4Gene Mutations: Pathophysiology and Clinical Aspects

Spraul, A., Gonzales, E. M., Baussan, C., & Jacquemin, E. (2010) · Seminars in Liver Disease · doi:10.1055/s-0030-1253223 · Cited by 245

The role of notch signaling in the development of intrahepatic bile ducts

Kodama, Y., Hijikata, M., Kageyama, R., Shimotohno, K., & Chiba, T. (2004) · Gastroenterology · doi:10.1053/j.gastro.2004.09.004 · Cited by 240

Liver and kidney disease in ciliopathies

Gunay‐Aygun, M. (2009) · American Journal of Medical Genetics Part C Seminars in Medical Genetics · doi:10.1002/ajmg.c.30225 · Cited by 236

Morphological, molecular, and functional heterogeneity of cholangiocytes from normal rat liver

Alpini, G. D., Roberts, S. K., Kuntz, S., Ueno, Y., Gubba, S., Podila, P. V., LeSage, G., & LaRusso, N. F. (1996) · Gastroenterology · doi:10.1053/gast.1996.v110.pm8613073 · Cited by 236

Demographic, lifestyle, medical and familial factors associated with primary biliary cirrhosis

Corpechot, C., Chrétien, Y., Chazouillères, O., & Poupon, R. (2010) · Journal of Hepatology · doi:10.1016/j.jhep.2010.02.019 · Cited by 235

Genome-wide association meta-analysis for total serum bilirubin levels

Johnson, A. D., Kavousi, M., Smith, A. V., Chen, M., Dehghan, A., Aspelund, T., Lin, J.-P., van Duijn, C. M., Harris, T. B., Cupples, L. A., et al. (2009) · Human Molecular Genetics · doi:10.1093/hmg/ddp202 · Cited by 235

IL-2 receptor α−/− mice and the development of primary biliary cirrhosis

Wakabayashi, K., Lian, Z.-X., Moritoki, Y., Lan, R. Y., Tsuneyama, K., Chuang, Y.-H., Yang, G.-X., Ridgway, W., Ueno, Y., Ansari, A. A., et al. (2006) · Hepatology · doi:10.1002/hep.21385 · Cited by 233

Distinct Roles for CCR4 and CXCR3 in the Recruitment and Positioning of Regulatory T Cells in the Inflamed Human Liver

Oo, Y. H., Weston, C. J., Lalor, P. F., Curbishley, S. M., Withers, D. R., Reynolds, G. M., Shetty, S., Harki, J., Shaw, J. C., Eksteen, B., et al. (2010) · The Journal of Immunology · doi:10.4049/jimmunol.0901216 · Cited by 232

Genetic Labeling Does Not Detect Epithelial-to-Mesenchymal Transition of Cholangiocytes in Liver Fibrosis in Mice

Scholten, D., Österreicher, C. H., Scholten, A., Iwaisako, K., Gu, G., Brenner, D. A., & Kisseleva, T. (2010) · Gastroenterology · doi:10.1053/j.gastro.2010.05.005 · Cited by 231

Multiple TLRs Are Expressed in Human Cholangiocytes and Mediate Host Epithelial Defense Responses to Cryptosporidium parvum via Activation of NF-κB

Chen, X., O’Hara, S. P., Nelson, J. B., Splinter, P. L., Small, A. J., Tietz, P. S., LIMPER, A. H., & LaRusso, N. F. (2005) · The Journal of Immunology · doi:10.4049/jimmunol.175.11.7447 · Cited by 231

Sclerosing cholangitis: a focus on secondary causes

Abdalian, R., & Heathcote, J. E. (2006) · Hepatology · doi:10.1002/hep.21405 · Cited by 230

Effects of ursodeoxycholic acid on survival in patients with primary biliary cirrhosis

Lindor, K., Therneau, T., Jorgensen, R., Malinchoc, M., & Dickson, E. (1996) · Gastroenterology · doi:10.1053/gast.1996.v110.pm8613058 · Cited by 228

Liver Autoimmunity Triggered by Microbial Activation of Natural Killer T Cells

Mattner, J., Savage, P. B., Leung, P. S. C., Oertelt‐Prigione, S., Wang, V., Trivedi, O., Scanlon, S. T., Pendem, K., Teyton, L., Hart, J., et al. (2008) · Cell Host & Microbe · doi:10.1016/j.chom.2008.03.009 · Cited by 224

Apotopes and the biliary specificity of primary biliary cirrhosis†

Lleo, A., Selmi, C., Invernizzi, P., Podda, M., Coppel, R. L., Mackay, I. R., Gores, G. J., Ansari, A. A., Van de Water, J., & Gershwin, M. E. (2009) · Hepatology · doi:10.1002/hep.22736 · Cited by 222

Increased prevalence of primary biliary cirrhosis near Superfund toxic waste sites

Ala, A., Stanca, C. M., Bu-Ghanim, M., Ahmado, I., Branch, A. D., Schiano, T. D., Odin, J. A., & Bach, N. (2006) · Hepatology · doi:10.1002/hep.21076 · Cited by 222

Quantitative and functional analysis of PDC-E2–specific autoreactive cytotoxic T lymphocytes in primary biliary cirrhosis

Kita, H., Matsumura, S., He, X.-S., Ansari, A. A., Lian, Z., de Water, J. V., Coppel, R. L., Kaplan, M. M., & Gershwin, M. E. (2002) · Journal of Clinical Investigation · doi:10.1172/jci14698 · Cited by 222

Quantitative and functional analysis of PDC‐E2‐specific autoreactive cytotoxic T lymphocytes in primary biliary cirrhosis

Kita, H., Matsumura, S., He, X.-S., Ansari, A. A., Lian, Z.-X., Van de Water, J., Coppel, R. L., Kaplan, M. M., & Gershwin, M. E. (2002) · Journal of Clinical Investigation · doi:10.1172/jci0214698 · Cited by 222

Bcl-2–dependent oxidation of pyruvate dehydrogenase-E2, a primary biliary cirrhosis autoantigen, during apoptosis

Odin, J. A., Huebert, R. C., Casciola-Rosen, L., LaRusso, N. F., & Rosen, A. (2001) · Journal of Clinical Investigation · doi:10.1172/jci200110716 · Cited by 220

Mechanisms of Cholestasis

Zollner, G., & Trauner, M. A. (2008) · Clinics in Liver Disease · doi:10.1016/j.cld.2007.11.010 · Cited by 219

Combined analysis of the effect of treatment with ursodeoxycholic acid on histologic progression in primary biliary cirrhosis

Poupon, R. E., Lindor, K. D., Parés, A. R., Chazouillères, O., Poupon, R. Y., & Heathcote, E. J. (2003) · Journal of Hepatology · doi:10.1016/s0168-8278(03)00192-2 · Cited by 216

Variants at IRF5-TNPO3, 17q12-21 and MMEL1 are associated with primary biliary cirrhosis

Hirschfield, G. M., Liu, X., Han, Y., Gorlov, I. P., Lu, Y., Xu, C., Lu, Y., Chen, W., Juran, B. D., Coltescu, C., et al. (2010) · Nature Genetics · doi:10.1038/ng.631 · Cited by 215

Biliary Apotopes and Anti-Mitochondrial Antibodies Activate Innate Immune Responses in Primary Biliary Cirrhosis†

Lleò, A., Bowlus, C. L., Yang, G., Invernizzi, P., Podda, M., de Water, J. V., Ansari, A. A., Coppel, R. L., Worman, H. J., Gores, G. J., et al. (2010) · Hepatology · doi:10.1002/hep.23783 · Cited by 215

Primary biliary cirrhosis once rare, now common in the United Kingdom?

James, O. F., Bhopal, R., Howel, D., Gray, J., Burt, A. D., & Metcalf, J. V. (1999) · Hepatology · doi:10.1002/hep.510300213 · Cited by 213

The Pathophysiology of Biliary Epithelia

Alpini, G., Alvaro, D., Marzioni, M., Lesage, G., & LaRusso, N. (2004) · CRC Press eBooks · doi:10.1201/9780367813888 · Cited by 212

Cholangiocyte proliferation and liver fibrosis

Glaser, S. S., Gaudio, E., Miller, T. J., Alvaro, D., & Alpini, G. D. (2009) · Expert Reviews in Molecular Medicine · doi:10.1017/s1462399409000994 · Cited by 207

Decreased Anion Exchanger 2 Immunoreactivity in the Liver of Patients With Primary Biliary Cirrhosis

Medina, J. F., Martínez‐Ansó, E., Vázquez, J. J., & Prìeto, J. (1997) · Hepatology · doi:10.1002/hep.510250104 · Cited by 206

Inhibition of Integrin αvβ6 on Cholangiocytes Blocks Transforming Growth Factor-β Activation and Retards Biliary Fibrosis Progression

Patsenker, E., Popov, Y. V., Stickel, F., Jonczyk, A., Goodman, S. L., & Schuppan, D. (2008) · Gastroenterology · doi:10.1053/j.gastro.2008.04.009 · Cited by 205

Hepatic IL-17 responses in human and murine primary biliary cirrhosis

Lan, R. Y. Z., Salunga, T. L., Tsuneyama, K., Lian, Z.-X., Yang, G.-X., Hsu, W., Moritoki, Y., Ansari, A. A., Kemper, C., Price, J., et al. (2009) · Journal of Autoimmunity · doi:10.1016/j.jaut.2008.11.001 · Cited by 202

Ae2-Deficient Mice Develop Antimitochondrial Antibodies and Other Features Resembling Primary Biliary Cirrhosis

Salas, J. T., Bañales, J. M., Sarvide, S., Recalde, S., Ferrer, A., Uriarte, I., Elferink, R. P. J. O., Prìeto, J., & Medina, J. F. (2008) · Gastroenterology · doi:10.1053/j.gastro.2008.02.020 · Cited by 196

Chemical Xenobiotics and Mitochondrial Autoantigens in Primary Biliary Cirrhosis: Identification of Antibodies against a Common Environmental, Cosmetic, and Food Additive, 2-Octynoic Acid

Amano, K., Leung, P. S. C., Rieger, R., Quan, C., Wang, X., Marik, J., Suen, Y. F., Kurth, M. J., Nantz, M. H., Ansari, A. A., et al. (2005) · The Journal of Immunology · doi:10.4049/jimmunol.174.9.5874 · Cited by 187

Secretory IgA: Arresting Microbial Pathogens at Epithelial Borders

Mantis, N. J., & Forbes, S. J. (2010) · Immunological Investigations · doi:10.3109/08820131003622635 · Cited by 185

Altered Monocyte Responses to Defined TLR Ligands in Patients With Primary Biliary Cirrhosis *

Mao, T. K., Lian, Z.-X., Selmi, C., Ichiki, Y., Ashwood, P., Ansari, A. A., Coppel, R. L., Shimoda, S., Ishibashi, H., & Gershwin, E. M. (2005) · Hepatology · doi:10.1002/hep.20859 · Cited by 185

Mutations in VIPAR cause an arthrogryposis, renal dysfunction and cholestasis syndrome phenotype with defects in epithelial polarization

Cullinane, A. R., Straatman-Iwanowska, A. A., Zaucker, A., Wakabayashi, Y., Bruce, C. K., Luo, G., Rahman, F., Gürakan, F., Ütine, E., Özkan, T., et al. (2010) · Nature Genetics · doi:10.1038/ng.538 · Cited by 184

Liver disease associated with canalicular transport defects: Current and future therapies

Stapelbroek, J. M., van Erpecum, K. J., Klomp, L. W. J., & Houwen, R. H. J. (2009) · Journal of Hepatology · doi:10.1016/j.jhep.2009.11.012 · Cited by 180

Periductal interleukin-17 production in association with biliary innate immunity contributes to the pathogenesis of cholangiopathy in primary biliary cirrhosis

Harada, K., Shimoda, S., Sato, Y., Isse, K., Ikeda, H., & Nakanuma, Y. (2009) · Clinical & Experimental Immunology · doi:10.1111/j.1365-2249.2009.03947.x · Cited by 179

NOD.c3c4 congenic mice develop autoimmune biliary disease that serologically and pathogenetically models human primary biliary cirrhosis

Irie, J., Wu, Y., Wicker, L. S., Rainbow, D. B., Nalesnik, M. A., Hirsch, R., Peterson, L. B., Leung, P. S. C., Cheng, C., Mackay, I. R., et al. (2006) · The Journal of Experimental Medicine · doi:10.1084/jem.20051911 · Cited by 179

The bile acid sensor farnesoid X receptor is a modulator of liver immunity in a rodent model of acute hepatitis

Mencarelli, A., Renga, B., Migliorati, M., Cipriani, S., Distrutti, E., Santucci, L., & Fiorucci, S. (2009) · The Journal of Immunology · doi:10.4049/jimmunol.0901347 · Cited by 177

Identification of 2-nonynoic acid, a cosmetic component, as a potential trigger of primary biliary cirrhosis

Rieger, R., Leung, P. S. C., Jeddeloh, M. R., Kurth, M. J., Nantz, M. H., Lam, K. S., Barsky, D., Ansari, A. A., Coppel, R. L., Mackay, I. R., et al. (2006) · Journal of Autoimmunity · doi:10.1016/j.jaut.2006.06.002 · Cited by 171

Case‐control studies of risk factors for primary biliary cirrhosis in two United Kingdom populations

Prince, M. I., Ducker, S. J., & James, O. F. W. (2010) · Gut · doi:10.1136/gut.2009.184218 · Cited by 171

Cholangiocyte primary cilia are chemosensory organelles that detect biliary nucleotides via P2Y 12 purinergic receptors

Masyuk, A. I., Gradilone, S. A., Banales, J. M., Huang, B. Q., Masyuk, T. V., Lee, S.-O., Splinter, P. L., Stroope, A. J., & LaRusso, N. F. (2008) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.90265.2008 · Cited by 170

Farnesoid X receptor critically determines the fibrotic response in mice but is expressed to a low extent in human hepatic stellate cells and periductal myofibroblasts

Fickert, P., Fuchsbichler, A., Moustafa, T., Wagner, M., Zollner, G., Halilbasic, E., Stöger, U., Arrese, M., Pizarro, M., Solís, N., et al. (2009) · The American Journal of Pathology · doi:10.2353/ajpath.2009.090114 · Cited by 169

The geographical distribution of primary biliary cirrhosis in a well-defined cohort

Prince, M. I., Chetwynd, A., Diggle, P., Jarner, M., Metcalf, J. V., & James, O. F. W. (2001) · Hepatology · doi:10.1053/jhep.2001.29760 · Cited by 166

The challenges in primary sclerosing cholangitis – Aetiopathogenesis, autoimmunity, management and malignancy

Weismüller, T. J., Wedemeyer, J., Kubicka, S., Strassburg, C. P., & Manns, M. P. (2008) · Journal of Hepatology · doi:10.1016/j.jhep.2008.01.020 · Cited by 163

Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2 −/− mice†‡§

Halilbasic, E., Fiorotto, R., Fickert, P., Marschall, H., Moustafa, T., Spirlı̀, C., Fuchsbichler, A., Gumhold, J., Silbert, D., Zatloukal, K., et al. (2009) · Hepatology · doi:10.1002/hep.22891 · Cited by 157

Large but not small intrahepatic bile ducts are involved in secretin-regulated ductal bile secretion

Alpini, G. D., Glaser, S. S., Robertson, W. E., Rodgers, R., Phinizy, J. L., Lasater, J., & LeSage, G. (1997) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.1997.272.5.g1064 · Cited by 154

p-ANCAs in autoimmune liver disorders recognise human β-tubulin isotype 5 and cross-react with microbial protein FtsZ

Terjung, B., Söhne, J., Lechtenberg, B., Gottwein, J. M., Muennich, M., Herzog, V., Mähler, M., Sauerbruch, T., & Spengler, U. (2009) · Gut · doi:10.1136/gut.2008.157818 · Cited by 153

Functional Anatomy of Normal Bile Ducts

Strazzabosco, M., & Fabris, L. (2008) · The Anatomical Record · doi:10.1002/ar.20664 · Cited by 148

Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease

Takeda, K., Kojima, Y., Ikejima, K., Harada, K., Yamashina, S., Okumura, K., Aoyama, T., Frese, S., Ikeda, H., Haynes, N. M., et al. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0802702105 · Cited by 148

Cyclic AMP Regulates Bicarbonate Secretion in Cholangiocytes Through Release of ATP Into Bile

Minagawa, N., Nagata, J., Shibao, K., Masyuk, A. I., Gomes, D. A., Rodrigues, M. Â., LeSage, G., Akiba, Y., Kaunitz, J. D., Ehrlich, B. E., et al. (2007) · Gastroenterology · doi:10.1053/j.gastro.2007.08.020 · Cited by 144

Bacterial CpG induces hyper-IgM production in CD27+ memory B cells in primary biliary cirrhosis

Kikuchi, K., Lian, Z., Yang, G., Ansari, A. A., Ikehara, S., Kaplan, M., Miyakawa, H., Coppel, R. L., & Gershwin, M. E. (2005) · Gastroenterology · doi:10.1053/j.gastro.2004.11.005 · Cited by 141

Preferential X chromosome loss but random inactivation characterize primary biliary cirrhosis†

Miozzo, M. R., Selmi, C. F., Gentilin, B., Grati, F. R., Sirchia, S. M., Oertelt‐Prigione, S., Zuin, M. G., Gershwin, M. E., Podda, M., & Invernizzi, P. (2007) · Hepatology · doi:10.1002/hep.21696 · Cited by 136

HLA class II alleles, genotypes, haplotypes, and amino acids in primary biliary cirrhosis: A large-scale study

Donaldson, P. T., Baragiotta, A. M., Heneghan, M. A., Floreani, A., Venturi, C., Underhill, J. A., Jones, D. E. J., James, O., & Bassendine, M. F. (2006) · Hepatology · doi:10.1002/hep.21316 · Cited by 136

Epidemiology and natural history of primary biliary cirrhosis in a Canadian health region: A population‐based study†

Myers, R. P., Shaheen, A. A. M., Fong, A., Burak, K. W., Wan, A., Swain, M. G., Hilsden, R. J., Sutherland, L., & Quan, H. (2009) · Hepatology · doi:10.1002/hep.23210 · Cited by 135

Assessment of biliary bicarbonate secretion in humans by positron emission tomography

Prìeto, J., García, N., Martí-Climent, J. M., Peñuelas, I., Richter, J. Á., & Medina, J. F. (1999) · Gastroenterology · doi:10.1016/s0016-5085(99)70564-0 · Cited by 135

Immunohistochemical Characterization of Hepatic Lymphocytes in Primary Biliary Cirrhosis in Comparison With Primary Sclerosing Cholangitis and Autoimmune Chronic Active Hepatitis

Hashimoto, E., Lindor, K. D., Homburger, H. A., Dickson, E. R., Czaja, A. J., Wiesner, R. H., & Ludwig, J. (1993) · Mayo Clinic Proceedings · doi:10.1016/s0025-6196(12)60897-0 · Cited by 132

Gut Homing Receptors on CD8 T Cells Are Retinoic Acid Dependent and Not Maintained by Liver Dendritic or Stellate Cells

Eksteen, B., Mora, J. R., Shepherd, E. L., Henderson, N. C., Lee–Turner, L., Villablanca, E. J., Curbishley, S. M., Aspinall, A. I., von Andrian, U. H., & Adams, D. (2009) · Gastroenterology · doi:10.1053/j.gastro.2009.02.046 · Cited by 131

Human leukocyte antigen polymorphisms in italian primary biliary cirrhosis: A multicenter study of 664 patients and 1992 healthy controls

Invernizzi, P., Selmi, C. F., Poli, F., Frison, S., Floreani, A., Alvaro, D., Almasio, P. L., Rosina, F., Marzioni, M., Fabris, L., et al. (2008) · Hepatology · doi:10.1002/hep.22567 · Cited by 130

The membrane-bound bile acid receptor TGR5 (Gpbar-1) is localized in the primary cilium of cholangiocytes

Keitel, V., Ullmer, C., & Häussinger, D. (2010) · Biological Chemistry · doi:10.1515/bc.2010.077 · Cited by 129

Heterogeneity of autoreactive T cell clones specific for the E2 component of the pyruvate dehydrogenase complex in primary biliary cirrhosis.

de Water, J. V., Ansari, A. A., Prindiville, T. P., Coppel, R. L., Ricalton, N. S., Kotzin, B. L., Liu, S., Roche, T. E., Krams, S. M., Muñoz, S., et al. (1995) · The Journal of Experimental Medicine · doi:10.1084/jem.181.2.723 · Cited by 128

Loss of Inositol 1,4,5-trisphosphate receptors from bile duct epithelia is a common event in cholestasis

Shibao, K., Hirata, K., Robert, M. E., & Nathanson, M. H. (2003) · Gastroenterology · doi:10.1016/s0016-5085(03)01201-0 · Cited by 125

Lymphocyte recruitment and homing to the liver in primary biliary cirrhosis and primary sclerosing cholangitis

Borchers, A. T., Shimoda, S., Bowlus, C. L., Keen, C. L., & Gershwin, M. E. (2009) · Seminars in Immunopathology · doi:10.1007/s00281-009-0167-2 · Cited by 125

High frequency of autoantibodies in patients with primary sclerosing cholangitis that bind biliary epithelial cells and induce expression of CD44 and production of interleukin 6

Xu, B., Broomé, U., Ericzon, B.-G., & Sumitran–Holgersson, S. (2002) · Gut · doi:10.1136/gut.51.1.120 · Cited by 124

Antimitochondrial antibodies in acute liver failure: implications for primary biliary cirrhosis

Leung, P. S. C., Rossaro, L., Davis, P. A., Park, O., Tanaka, A., Kikuchi, K., Miyakawa, H., Norman, G. L., Lee, W., & Gershwin, E. M. (2007) · Hepatology · doi:10.1002/hep.21828 · Cited by 121

Genetic factors of susceptibility and of severity in primary biliary cirrhosis

Poupon, R. Y., Chen, P., Chrétien, Y., Corpechot, C., Chazouillères, O., Simon, T., Heath, S. C., Matsuda, F., Poupon, R. E., Housset, C., et al. (2008) · Journal of Hepatology · doi:10.1016/j.jhep.2008.07.027 · Cited by 121

CD40 activation‐induced, Fas‐dependent apoptosis and NF‐κB/AP‐1 signaling in human intrahepatic biliary epithelial cells

Afford, S. C., Ahmed-Choudhury, J., Randhawa, S., Russell, C. L., Youster, J., Crosby, H. A., Eliopoulos, A. G., Hübscher, S. G., Young, L. S., & Adams, D. (2001) · The FASEB Journal · doi:10.1096/fj.01-0088com · Cited by 118

Update on primary sclerosing cholangitis

Karlsen, T. H., Schrumpf, E., & Boberg, K. M. (2010) · Digestive and Liver Disease · doi:10.1016/j.dld.2010.01.011 · Cited by 117

Deletion of interleukin-12p40 suppresses autoimmune cholangitis in dominant negative transforming growth factor β receptor type II mice #

Yoshida, K., Yang, G., Zhang, W., Tsuda, M., Tsuneyama, K., Moritoki, Y., Ansari, A. A., Okazaki, K., Lian, Z., Coppel, R. L., et al. (2009) · Hepatology · doi:10.1002/hep.23132 · Cited by 116

Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells

Arenas, F., Hervías, I., Úriz, M., Joplin, R., Prìeto, J., & Medina, J. F. (2008) · Journal of Clinical Investigation · doi:10.1172/jci33156 · Cited by 116

Intracellular pH regulation in isolated rat bile duct epithelial cells.

Strazzabosco, M., Mennone, A., & Boyer, J. L. (1991) · Journal of Clinical Investigation · doi:10.1172/jci115160 · Cited by 112

Ursodeoxycholic Acid Stimulates Cholangiocyte Fluid Secretion in Mice via CFTR-Dependent ATP Secretion

Fiorotto, R., Spirlı̀, C., Fabris, L., Cadamuro, M., Okolicsànyi, L., & Strazzabosco, M. (2007) · Gastroenterology · doi:10.1053/j.gastro.2007.08.071 · Cited by 111

The immunobiology of primary sclerosing cholangitis

Aron, J. H., & Bowlus, C. L. (2009) · Seminars in Immunopathology · doi:10.1007/s00281-009-0154-7 · Cited by 110

The Bile Salt Export Pump: Clinical and Experimental Aspects of Genetic and Acquired Cholestatic Liver Disease

Lam, P., Soroka, C. J., & Boyer, J. L. (2010) · Seminars in Liver Disease · doi:10.1055/s-0030-1253222 · Cited by 109

Cx3cl1 (Fractalkine): A Signpost for Biliary Inflammation in Primary Biliary Cirrhosis

Shimoda, S., Harada, K., Niiro, H., Taketomi, A., Maehara, Y., Tsuneyama, K., Kikuchi, K., Nakanuma, Y., Mackay, I. R., Gershwin, E. M., et al. (2010) · Hepatology · doi:10.1002/hep.23318 · Cited by 108

Defective regulation of cholangiocyte Cl−/HCO− 3 and Na+/H+ exchanger activities in primary biliary cirrhosis

Melero, S., Spirlì, C., Zsembery, Á., Medina, J. F., Joplin, R. E., Duner, E., Zuin, M., Neuberger, J. M., Prieto, J., & Strazzabosco, M. (2002) · Hepatology · doi:10.1053/jhep.2002.33634 · Cited by 107

Assessment of Biliary Bicarbonate Secretion in Humans by Positron Emission Tomography

Prìeto, J., García, N., Martí-Climent, J. M., Peñuelas, L., Richter, J. Á., & Medina, J. F. (2017) · Revista de medicina de la Universidad de Navarra · doi:10.15581/021.6810 · Cited by 106

Mapping MHC-encoded susceptibility and resistance in primary sclerosing cholangitis: The role of MICA polymorphism

Norris, S., Kondeatis, E., Collins, R., Stephens, H. A. F., Harrison, P., Vaughan, R., Clare, M., Satsangi, J. J., Chapman, R. W., & Donaldson, P. T. (2001) · Gastroenterology · doi:10.1053/gast.2001.24041 · Cited by 103

Nuclear Receptor Regulation of the Adaptive Response of Bile Acid Transporters in Cholestasis

Wagner, M., Zollner, G., & Trauner, M. A. (2010) · Seminars in Liver Disease · doi:10.1055/s-0030-1253225 · Cited by 103

Phylogenetic and immunological definition of four lipoylated proteins from Novosphingobium aromaticivorans, implications for primary biliary cirrhosis

Padgett, K. A., Selmi, C. F., Kenny, T. P., Leung, P. S. C., Balkwill, D. L., Ansari, A. A., Coppel, R. L., & Gershwin, M. E. (2005) · Journal of Autoimmunity · doi:10.1016/j.jaut.2005.01.012 · Cited by 102

Particular genetic variants of ligands for natural killer cell receptors may contribute to the HLA associated risk of primary sclerosing cholangitis

Karlsen, T. H., Boberg, K. M., Olsson, M., Sun, J., Senitzer, D., Bergquist, A. M., Schrumpf, E., Thorsby, E., & Lie, B. A. (2007) · Journal of Hepatology · doi:10.1016/j.jhep.2007.01.032 · Cited by 92

CD4 T-cell autoreactivity to the mitochondrial autoantigen PDC-E2 in AMA-negative primary biliary cirrhosis

Shimoda, S., Miyakawa, H., Nakamura, M., Ishibashi, H., Kikuchi, K., Kita, H., Niiro, H., Arinobu, Y., Ono, N., Mackay, I. R., et al. (2008) · Journal of Autoimmunity · doi:10.1016/j.jaut.2008.05.003 · Cited by 92

Nuclear receptors as drug targets in cholestasis and drug-induced hepatotoxicity

Zollner, G., Wagner, M., & Trauner, M. (2010) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2010.03.005 · Cited by 91

IKK1 and IKK2 cooperate to maintain bile duct integrity in the liver

Luedde, T., Heinrichsdorff, J., Lorenzi, R. D., Vos, R. D., Roskams, T., & Pasparakis, M. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0800198105 · Cited by 90

Cholangiociliopathies: genetics, molecular mechanisms and potential therapies

Masyuk, T. V., Masyuk, A. I., & LaRusso, N. F. (2009) · Current Opinion in Gastroenterology · doi:10.1097/mog.0b013e328328f4ff · Cited by 89

Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis – Overlapping or separate diseases?

Webster, G. J. M., Pereira, S. P., & Chapman, R. W. (2009) · Journal of Hepatology · doi:10.1016/j.jhep.2009.04.010 · Cited by 89

Primary sclerosing cholangitis is associated to an extended B8-DR3 haplotype including particular MICA and MICB alleles

Wiencke, K., Spurkland, A., Schrumpf, E., & Boberg, K. M. (2001) · Hepatology · doi:10.1053/jhep.2001.27543 · Cited by 89

Knockout of Secretin Receptor Reduces Large Cholangiocyte Hyperplasia in Mice With Extrahepatic Cholestasis Induced by Bile Duct Ligation

Glaser, S. S., Lam, I. P. Y., Franchitto, A., Gaudio, E., Onori, P., Chow, B., Wise, C., Kopriva, S. E., Venter, J. K., White, M., et al. (2010) · Hepatology · doi:10.1002/hep.23657 · Cited by 89

Portal hypertension and primary biliary cirrhosis: effect of long-term ursodeoxycholic acid treatment

Huet, P., Vincent, C., Deslaurier, J., Coté, J., Matsutami, S., Boileau, R., & Huet–van Kerckvoorde, J. (2008) · Gastroenterology · doi:10.1053/j.gastro.2008.07.019 · Cited by 84

Immunogenicity of Biliary Epithelium: Investigation of Antigen Presentation to Cd4+ T Cells

Leon, M. P., Bassendine, M. F., Wilson, J. L., Ali, S., Thick, M., & Kirby, J. A. (1996) · Hepatology · doi:10.1002/hep.510240317 · Cited by 83

The immunobiology of cholangiocytes

Chen, X., O’Hara, S. P., & LaRusso, N. F. (2008) · Immunology and Cell Biology · doi:10.1038/icb.2008.37 · Cited by 83

AMA production in primary biliary cirrhosis is promoted by the TLR9 ligand CpG and suppressed by potassium channel blockers

Moritoki, Y., Lian, Z., Wulff, H., Yang, G., Chuang, Y., Lan, R. Y., Ueno, Y., Ansari, A. A., Coppel, R. L., Mackay, I. R., et al. (2007) · Hepatology · doi:10.1002/hep.21522 · Cited by 80

B Cells Suppress the Inflammatory Response in a Mouse Model of Primary Biliary Cirrhosis

Moritoki, Y., Zhang, W., Tsuneyama, K., Yoshida, K., Wakabayashi, K., Yang, G., Bowlus, C. L., Ridgway, W. M., Ueno, Y., Ansari, A. A., et al. (2008) · Gastroenterology · doi:10.1053/j.gastro.2008.11.035 · Cited by 80

CCL25 and CCL28 promote α 4 β 7 -integrin-dependent adhesion of lymphocytes to MAdCAM-1 under shear flow

Miles, A., Liaskou, E., Eksteen, B., Lalor, P. F., & Adams, D. (2008) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00266.2007 · Cited by 79

Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization

Wakabayashi, K., Yoshida, K., Leung, P. S. C., Moritoki, Y., Yang, G.-X., Tsuneyama, K., Lian, Z.-X., Hibi∥, T., Ansari, A. A., Wicker, L. S., et al. (2008) · Clinical & Experimental Immunology · doi:10.1111/j.1365-2249.2008.03837.x · Cited by 78

Expression and cytokine regulation of immune recognition elements by normal human biliary epithelial and established liver cell lines in vitro

Cruickshank, S. M., Southgate, J., Selby, P. J., & Trejdosiewicz, L. K. (1998) · Journal of Hepatology · doi:10.1016/s0168-8278(98)80149-9 · Cited by 77

Induction of colitis in cftr−/− mice results in bile duct injury

Blanco, P. G., Zaman, M. M., Junaidi, O., Sheth, S. G., Yantiss, R. K., Nasser, I. A., & Freedman, S. D. (2004) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00452.2003 · Cited by 75

The role of environmental factors in primary biliary cirrhosis

Selmi, C. F., & Gershwin, M. E. (2009) · Trends in Immunology · doi:10.1016/j.it.2009.05.006 · Cited by 74

Medical Management of Alagille Syndrome

Kamath, B. M., Loomes, K. M., & Piccoli, D. A. (2010) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0b013e3181d98ea8 · Cited by 74

Are Transient Environmental Agents Involved in the Cause of Primary Biliary Cirrhosis? Evidence from Space–Time Clustering Analysis†

McNally, R. J. Q., Ducker, S., & James, O. F. W. (2009) · Hepatology · doi:10.1002/hep.23139 · Cited by 72

Immunohistochemical analysis of inflammation in primary sclerosing cholangitis

Ponsioen, C. Y., Kuiper, H., ten Kate, F. J. W., de Wit, M. V. M., van Deventer, S. J. H., & Tytgat, G. N. (1999) · European Journal of Gastroenterology & Hepatology · doi:10.1097/00042737-199907000-00015 · Cited by 71

Immunology of the gut and liver: a love/hate relationship

Adams, D., Eksteen, B., & Curbishley, S. M. (2008) · Gut · doi:10.1136/gut.2007.122168 · Cited by 71

Innate Immunity and Primary Biliary Cirrhosis

Selmi, C. F., Lleò, A., Pasini, S. M., Zuin, M. G., & Gershwin, M. E. (2009) · Current Molecular Medicine · doi:10.2174/156652409787314525 · Cited by 65

Experimental evidence on the immunopathogenesis of primary biliary cirrhosis

Selmi, C. F., Meda, F., Kasangian, A. A., Invernizzi, P., Tian, Z., Lian, Z., Podda, M., & Gershwin, M. E. (2009) · Cellular and Molecular Immunology · doi:10.1038/cmi.2009.104 · Cited by 63

Immunogenicity of biliary epithelium: Study of the adhesive interaction with lymphocytes

Leon, M. P., Bassendine, M. F., Gibbs, P., Thick, M., & Kirby, J. A. (1997) · Gastroenterology · doi:10.1053/gast.1997.v112.pm9041260 · Cited by 59

Diferentially expressed adenylyl cyclase isoforms mediate secretory functions in cholangiocyte subpopulation #

Strazzabosco, M., Fiorotto, R., Melero, S., Glaser, S. S., Francis, H., Spirlı̀, C., & Alpini, G. D. (2009) · Hepatology · doi:10.1002/hep.22926 · Cited by 59

Coculture of human liver macrophages and cholangiocytes leads to CD40-dependent apoptosis and cytokine secretion

Alabraba, E., Lai, V., Boon, L., Wigmore, S. J., Adams, D., & Afford, S. C. (2007) · Hepatology · doi:10.1002/hep.22011 · Cited by 58

Pathology of Immune-Mediated Liver Injury

Dienes, H., & Drebber, U. (2010) · Digestive Diseases · doi:10.1159/000282065 · Cited by 57

Genetic associations in Italian primary sclerosing cholangitis: Heterogeneity across Europe defines a critical role for HLA-C

Hov, J. R., Lleò, A., Selmi, C. F., Woldseth, B., Fabris, L., Strazzabosco, M., Karlsen, T. H., & Invernizzi, P. (2010) · Journal of Hepatology · doi:10.1016/j.jhep.2009.11.029 · Cited by 56

Antimitochondrial Antibodies and Reactivity to N. Aromaticivorans Proteins in Icelandic Patients with Primary Biliary Cirrhosis and Their Relatives

Ólafsson, S., Gudjónsson, H., Selmi, C. F., Amano, K., Invernizzi, P., Podda, M., & Gershwin, M. E. (2004) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.2004.40397.x · Cited by 49

Quality of life and everyday activities in patients with primary biliary cirrhosis

Selmi, C. F., Gershwin, M. E., Lindor, K. D., Worman, H. J., Gold, E. B., Watnik, M., Utts, J. M., Invernizzi, P., Kaplan, M. M., Vierling, J. M., et al. (2007) · Hepatology · doi:10.1002/hep.21953 · Cited by 46

Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis

Henckaerts, L., Jaspers, M., Steenbergen, W. V., Vliegen, L., Fevery, J., Nuytten, H., Roskams, T., Rutgeerts, P. J., Cassiman, J., Vermeire, S., et al. (2008) · Journal of Hepatology · doi:10.1016/j.jhep.2008.07.033 · Cited by 45

Helicobacter pylori May Play a Contributory Role in the Pathogenesis of Primary Sclerosing Cholangitis

Krasinskas, A. M., Yao, Y., Randhawa, P. S., Dore, M. P., & Sepulveda, A. R. (2007) · Digestive Diseases and Sciences · doi:10.1007/s10620-007-9803-7 · Cited by 45

The immunopathology of human biliary cell epithelium

Chuang, Y., Lan, R. Y., & Gershwin, M. E. (2009) · Seminars in Immunopathology · doi:10.1007/s00281-009-0172-5 · Cited by 43

Naturally-occurring regulatory T cells are increased in inflamed portal tracts with cholangiopathy in primary biliary cirrhosis

Sasaki, M., Ikeda, H., Sawada, S., Sato, Y., & Nakanuma, Y. (2006) · Journal of Clinical Pathology · doi:10.1136/jcp.2006.044776 · Cited by 40

Anomalous expression of costimulatory molecules B7-1, B7-2 and CD28 in primary biliary cirrhosis

Spengler, U., Leifeld, L., Braunschweiger, I., Dumoulin, F. L., Lechmann, M., & Sauerbruch, T. (1997) · Journal of Hepatology · doi:10.1016/s0168-8278(97)80006-2 · Cited by 39

Pathobiology of biliary epithelia and cholangiocarcinoma: Proceedings of the Henry M. and Lillian Stratton basic research single-topic conference

Sirica, A. E., Nathanson, M. H., Gores, G. J., & LaRusso, N. F. (2008) · Hepatology · doi:10.1002/hep.22623 · Cited by 36

New Insights into Autoimmune Cholangitis through Animal Models

Trauner, M., Fickert, P., Baghdasaryan, A., Claudel, T., Halilbasic, E., Moustafa, T., Wagner, M., & Zollner, G. (2010) · Digestive Diseases · doi:10.1159/000282072 · Cited by 35

Murine models of autoimmune cholangitis

Ueno, Y., Ambrosini, Y. M., Moritoki, Y., Ridgway, W. M., & Gershwin, M. E. (2010) · Current Opinion in Gastroenterology · doi:10.1097/mog.0b013e32833755aa · Cited by 32

Control of Cholangiocyte Adaptive Responses by Visceral Hormones and Neuropeptides

Marzioni, M., Fava, G., Alvaro, D., Alpini, G. D., & Benedetti, A. (2008) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-008-8090-1 · Cited by 32

Differential expression of intestinal trefoil factor in biliary epithelial cells of primary biliary cirrhosis

Kimura, Y., Leung, P. S. C., Kenny, T. P., de Water, J. V., Nishioka, M., Giraud, A. S., Neuberger, J., Benson, G. D., Kaul, R., Ansari, A. A., et al. (2002) · Hepatology · doi:10.1053/jhep.2002.36157 · Cited by 28

Common Genetic Variation and Haplotypes of the Anion Exchanger SLC4A2 in Primary Biliary Cirrhosis

Juran, B. D., Atkinson, E. J., Larson, J. J., Schlicht, E. M., & Lazaridis, K. N. (2009) · The American Journal of Gastroenterology · doi:10.1038/ajg.2009.103 · Cited by 22

Apoptosis in Immune-Mediated Liver Diseases

Kahraman, A., Gerken, G., & Canbay, A. E. (2010) · Digestive Diseases · doi:10.1159/000299799 · Cited by 20

Transient development of anti-mitochondrial antibodies accompanies autoimmune hepatitis–sclerosing cholangitis overlap

Bhat, M., Guindi, M. M., Heathcote, E. J., & Hirschfield, G. M. (2008) · Gut · doi:10.1136/gut.2008.163220 · Cited by 15

It's all about bile†

Boyer, J. L. (2009) · Hepatology · doi:10.1002/hep.22719 · Cited by 11

Old and rising stars in the lymphoid liver

Selmi, C. F., Podda, M., & Gershwin, M. E. (2009) · Seminars in Immunopathology · doi:10.1007/s00281-009-0175-2 · Cited by 6

The Canadian Multicenter Double‐blind Randomized Controlled Trial of ursodeoxycholic acid in primary biliary cirrhosis

Heathcote, J. E., Cauch-Dudek, K., Walker, V., Bailey, R. J., Blendis, L. M., Ghent, C. N., Michieletti, P., Minuk, G. Y., Pappas, C. S., Scully, L. J., et al. (1994) · Hepatology · doi:10.1002/hep.1840190512 · Cited by 2

Fibrates and cholestasis

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

Primary biliary cholangitis: pathogenesis and therapeutic opportunities

Gulamhusein, A. F., & Hirschfield, G. M. (2020) · Nature Reviews Gastroenterology & Hepatology · doi:10.1038/s41575-019-0226-7 · Cited by 308

Ellagic acid: Pharmacological activities and molecular mechanisms involved in liver protection

García‐Niño, W. R., & Zazueta, C. (2015) · Pharmacological Research · doi:10.1016/j.phrs.2015.04.008 · Cited by 279

Bile acids permeabilize the blood brain barrier after bile duct ligation in rats via Rac1-dependent mechanisms

Quinn, M., McMillin, M., Galindo, C., Frampton, G., Pae, H. Y., & DeMorrow, S. (2014) · Digestive and Liver Disease · doi:10.1016/j.dld.2014.01.159 · Cited by 259

The Spectrum of Achalasia: Lessons From Studies of Pathophysiology and High-Resolution Manometry

Kahrilas, P. J., & Boeckxstaens, G. E. (2013) · Gastroenterology · doi:10.1053/j.gastro.2013.08.038 · Cited by 236

Cholestasis and cholestatic syndromes

Hirschfield, G. M., & Heathcote, E. J. (2009) · Current Opinion in Gastroenterology · doi:10.1097/mog.0b013e32832914b4 · Cited by 27

Chronic cholestatic liver diseases: Clues from histopathology for pathogenesis

Pollheimer, M. J., Fickert, P., & Stieger, B. (2013) · Molecular Aspects of Medicine · doi:10.1016/j.mam.2013.10.001 · Cited by 46

Comprehensive Review of Molecular Mechanisms during Cholestatic Liver Injury and Cholangiocarcinoma

Virani, S., Akers, A. T., Stephenson, K., Smith, S. M., Kennedy, L. L., Alpini, G. D., & Francis, H. (2018) · Journal of Liver · doi:10.4172/2167-0889.1000231 · Cited by 9

Review: Pathogenesis of cholestatic liver diseases

Yokoda, R. T., & Rodríguez, E. A. (2020) · World Journal of Hepatology · doi:10.4254/wjh.v12.i8.423 · Cited by 72

New insights on the molecular and cell biology of human cholangiopathies

Alvaro, D., & Mancino, M. G. (2007) · Molecular Aspects of Medicine · doi:10.1016/j.mam.2007.09.007 · Cited by 46