Animal models of primary biliary cholangitis: status and challenges

Shimoda S, Tanaka A. It is time to change primary biliary cirrhosis (PBC): new nomenclature from “cirrhosis” to “cholangitis”, and upcoming treatment based on unveiling pathology. Hepatol Res. 2016;46(5):407–15. https://doi.org/10.1111/hepr.12615.

Article  PubMed  Google Scholar 

Trivedi PJ, Hirschfield GM. Recent advances in clinical practice: epidemiology of autoimmune liver diseases. Gut. 2021;70(10):1989–2003. https://doi.org/10.1136/gutjnl-2020-322362.

Article  PubMed  Google Scholar 

Zeng N, Duan W, Chen S, Wu S, Ma H, Ou X, et al. Epidemiology and clinical course of primary biliary cholangitis in the Asia-Pacific region: a systematic review and meta-analysis. Hepatol Int. 2019;13(6):788–99. https://doi.org/10.1007/s12072-019-09984-x.

Article  PubMed  Google Scholar 

Gerussi A, Cristoferi L, Carbone M, Asselta R, Invernizzi P. The immunobiology of female predominance in primary biliary cholangitis. J Autoimmun. 2018;95:124–32. https://doi.org/10.1016/j.jaut.2018.10.015.

Article  PubMed  Google Scholar 

Ellinghaus D. How genetic risk contributes to autoimmune liver disease. Semin Immunopathol. 2022;44(4):397–410. https://doi.org/10.1007/s00281-022-00950-8.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Matsumoto K, Ohfuji S, Abe M, Komori A, Takahashi A, Fujii H, et al. Environmental factors, medical and family history, and comorbidities associated with primary biliary cholangitis in Japan: a multicenter case-control study. J Gastroenterol. 2022;57(1):19–29. https://doi.org/10.1007/s00535-021-01836-6.

Article  PubMed  Google Scholar 

Ma WT, Chen DK. Immunological abnormalities in patients with primary biliary cholangitis. Clin Sci (Lond). 2019;133(6):741–60. https://doi.org/10.1042/cs20181123.

Article  CAS  PubMed  Google Scholar 

Chen Z, Liu S, He C, Sun J, Wang L, Chen H, et al. CXCL12-CXCR4-mediated chemotaxis supports accumulation of mucosal-associated invariant T cells into the liver of patients with PBC. Front Immunol. 2021;12: 578548. https://doi.org/10.3389/fimmu.2021.578548.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Schrumpf E, Tan C, Karlsen TH, Sponheim J, Björkström NK, Sundnes O, et al. The biliary epithelium presents antigens to and activates natural killer T cells. Hepatology. 2015;62(4):1249–59. https://doi.org/10.1002/hep.27840.

Article  CAS  PubMed  Google Scholar 

Li X, Li Y, Xiao J, Wang H, Guo Y, Mao X, et al. Unique DUOX2(+)ACE2(+) small cholangiocytes are pathogenic targets for primary biliary cholangitis. Nat Commun. 2023;14(1):29. https://doi.org/10.1038/s41467-022-34606-w.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Younossi ZM, Bernstein D, Shiffman ML, Kwo P, Kim WR, Kowdley KV, et al. diagnosis and management of primary biliary cholangitis. Am J Gastroenterol. 2019;114(1):48–63. https://doi.org/10.1038/s41395-018-0390-3.

Article  PubMed  Google Scholar 

Laschtowitz A, de Veer RC, Van der Meer AJ, Schramm C. Diagnosis and treatment of primary biliary cholangitis. United European Gastroenterol J. 2020;8(6):667–74. https://doi.org/10.1177/2050640620919585.

Article  PubMed  PubMed Central  Google Scholar 

Liu C-H, Bowlus CL. Treatment of Primary Biliary Cholangitis: First-Line and Second-Line Therapies. Clin Liver Dis. 2022;26(4):705–26. https://doi.org/10.1016/j.cld.2022.06.012.

Article  PubMed  Google Scholar 

Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D, Vierling JM, Adams D, Alpini G, et al. The challenges of primary biliary cholangitis: What is new and what needs to be done. J Autoimmun. 2019;105: 102328. https://doi.org/10.1016/j.jaut.2019.102328.

Article  PubMed  Google Scholar 

Liu S-P, Bian Z-H, Zhao Z-B, Wang J, Zhang W, Leung PSC, et al. Animal models of autoimmune liver diseases: a comprehensive review. Clin Rev Allergy Immunol. 2020;58(2):252–71. https://doi.org/10.1007/s12016-020-08778-6.

Article  PubMed  Google Scholar 

Itoh A, Adams D, Huang W, Wu Y, Kachapati K, Bednar KJ, et al. Enoxacin up-regulates MicroRNA biogenesis and down-regulates cytotoxic CD8 T-cell function in autoimmune cholangitis. Hepatology. 2021;74(2):835–46. https://doi.org/10.1002/hep.31724.

Article  CAS  PubMed  Google Scholar 

Kawata K, Yang G-X, Ando Y, Tanaka H, Zhang W, Kobayashi Y, et al. Clonality, activated antigen-specific CD8(+) T cells, and development of autoimmune cholangitis in dnTGFβRII mice. Hepatology. 2013;58(3):1094–104. https://doi.org/10.1002/hep.26418.

Article  CAS  PubMed  Google Scholar 

Gorelik L, Flavell RA. Abrogation of TGFbeta signaling in T cells leads to spontaneous T cell differentiation and autoimmune disease. Immunity. 2000;12(2):171–81. https://doi.org/10.1016/s1074-7613(00)80170-3.

Article  CAS  PubMed  Google Scholar 

Oertelt S, Lian Z-X, Cheng C-M, Chuang Y-H, Padgett KA, He X-S, et al. Anti-mitochondrial antibodies and primary biliary cirrhosis in TGF-beta receptor II dominant-negative mice. J Immunol. 2006;177(3):1655–60. https://doi.org/10.4049/jimmunol.177.3.1655.

Article  CAS  PubMed  Google Scholar 

Li Y, Li B, You Z, Zhang J, Wei Y, Li Y, et al. Cytotoxic KLRG1 expressing lymphocytes invade portal tracts in primary biliary cholangitis. J Autoimmun. 2019;103: 102293. https://doi.org/10.1016/j.jaut.2019.06.004.

Article  CAS  PubMed  Google Scholar 

Huang W, Kachapati K, Adams D, Wu Y, Leung PSC, Yang G-X, et al. Murine autoimmune cholangitis requires two hits: cytotoxic KLRG1(+) CD8 effector cells and defective T regulatory cells. J Autoimmun. 2014;50:123–34. https://doi.org/10.1016/j.jaut.2014.01.034.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Zhang W, Zhang R, Zhang J, Sun Y, Leung PS, Yang G-X, et al. Proteomic analysis reveals distinctive protein profiles involved in CD8+ T cell-mediated murine autoimmune cholangitis. Cell Mol Immunol. 2018;15(8):756–67. https://doi.org/10.1038/cmi.2017.149.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Han Y, Bian Z-H, Yang S-Y, Wang C-B, Li L, Yang Y-Q, et al. Single-cell characterization of hepatic CD8 T cells in a murine model of primary biliary cholangitis. Front Immunol. 2022;13: 860311. https://doi.org/10.3389/fimmu.2022.860311.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Ma H-D, Ma W-T, Liu Q-Z, Zhao Z-B, Liu M-Z-Y, Tsuneyama K, et al. Chemokine receptor CXCR3 deficiency exacerbates murine autoimmune cholangitis by promoting pathogenic CD8 T cell activation. J Autoimmun. 2017;78:19–28. https://doi.org/10.1016/j.jaut.2016.12.012.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Yang J-B, Wang Y-H, Yang W, Lu F-T, Ma H-D, Zhao Z-B, et al. Successful treatment of murine autoimmune cholangitis by parabiosis: implications for hematopoietic therapy. J Autoimmun. 2016;66:108–17. https://doi.org/10.1016/j.jaut.2015.09.002.

Article  CAS  PubMed  Google Scholar 

Zhao ZB, Lu FT, Ma HD, Wang YH, Yang W, Long J, et al. Liver-resident NK cells suppress autoimmune cholangitis and limit the proliferation of CD4(+) T cells. Cell Mol Immunol. 2020;17(2):178–89. https://doi.org/10.1038/s41423-019-0199-z.

Article  CAS  PubMed  Google Scholar 

Leung PSC, Choi J, Yang G, Woo E, Kenny TP, Gershwin ME. A contemporary perspective on the molecular characteristics of mitochondrial autoantigens and diagnosis in primary biliary cholangitis. Expert Rev Mol Diagn. 2016;16(6):697–705. https://doi.org/10.1586/14737159.2016.1164038.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Dhirapong A, Lleo A, Yang G-X, Tsuneyama K, Dunn R, Kehry M, et al. B cell depletion therapy exacerbates murine primary biliary cirrhosis. Hepatology. 2011;53(2):527–35. https://doi.org/10.1002/hep.24044.

Article  CAS  PubMed  Google Scholar 

Moritoki Y, Tsuneyama K, Nakamura Y, Kikuchi K, Shiota A, Ohsugi Y, et al. Anti-drug antibodies against a novel humanized anti-CD20 antibody impair its therapeutic effect on primary biliary cholangitis in human CD20- and FcgammaR-expressing mice. Front Immunol. 2018;9:2534. https://doi.org/10.3389/fimmu.2018.02534.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Kennedy L, Francis H, Invernizzi P, Venter J, Wu N, Carbone M, et al. Secretin/secretin receptor signaling mediates biliary damage and liver fibrosis in early-stage primary biliary cholangitis. FASEB J. 2019;33(9):10269–79. https://doi.org/10.1096/fj.201802606R.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Kennedy L, Carpino G, Owen T, Ceci L, Kundu D, Meadows V, et al. Secretin alleviates biliary and liver injury during late-stage primary biliary cholangitis via restoration of secretory processes. J Hepatol. 2023;78(1):99–113. https://doi.org/10.1016/j.jhep.2022.07.034.

Article  CAS  PubMed  Google Scholar 

Ma HD, Zhao ZB, Ma WT, Liu QZ, Gao CY, Li L, et al. Gut microbiota translocation promotes autoimmune cholangitis. J Autoimmun. 2018;95:47–57. https://doi.org/10.1016/j.jaut.2018.09.010.

Article  CAS 

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