Animal models of primary biliary cholangitis: status and challenges

被引：6

作者：

Wang, Xu ^{[1
]}

Wei, Yi ^{[1
]}

Yang, Yanlei ^{[2
]}

Yang, Yunjiao ^{[1
]}

Li, Haolong ^{[3
]}

Li, Yongzhe ^{[3
]}

Zhang, Fengchun ^{[1
]}

Wang, Li ^{[1
]}

机构：

[1] Chinese Acad Med Sci, Peking Union Med Coll Hosp, Peking Union Med Coll, Key Lab Rheumatol & Clin Immunol,Minist Educ,Dept, Beijing, Peoples R China

[2] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Med Res Cent, Clin Biobank, Beijing, Peoples R China

[3] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Clin Lab, Beijing, Peoples R China

来源：

CELL AND BIOSCIENCE | 2023年 / 13卷 / 01期

关键词：

Primary biliary cholangitis; Genetically modified models; Chemically inducible models; Biologically inducible models; Challenges; MURINE AUTOIMMUNE CHOLANGITIS; CD8(+) T-CELLS; ANTIMITOCHONDRIAL ANTIBODIES; C57BL/6; MICE; MOUSE MODEL; CIRRHOSIS; DIFFERENTIATION; CONTRIBUTES; DEFICIENCY; EXPRESSION;

D O I：

10.1186/s13578-023-01170-9

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

BackgroundPrimary biliary cholangitis (PBC) is an autoimmune liver disease. The aetiology of PBC remains unclear, and its pathogenesis is complex. Animal models are essential to clarify the pathogenesis of PBC and explore the occurrence of early events.Main bodyHerein, we review recent research progress in PBC animal models, including genetically modified, chemically inducible, biologically inducible, and protein-immunised models. Although these animal models exhibit several immunological and pathological features of PBC, they all have limitations that constrain further research and weaken their connection with clinical practice.ConclusionThe review will benefit efforts to understand and optimise animal models in order to further clarify PBC pathogenesis and molecular targets for therapeutic interventions.

引用

页数：10

共 86 条

[1] Promoter hypermethylation of the AE2/SLC4A2 gene in PBC [J].

Arenas, Fabian ;

Hervias, Isabel ;

Saez, Elena ;

Melero, Saida ;

Prieto, Jesus ;

Pares, Albert ;

Medina, Juan F. .

JHEP REPORTS, 2019, 1 (03) :145-153

[2] The interplay of type I and type II interferons in murine autoimmune cholangitis as a basis for sex-biased autoimmunity [J].

Bae, Heekyong R. ;

Hodge, Deborah L. ;

Yang, Guo-Xiang ;

Leung, Patrick S. C. ;

Chodisetti, Sathi Babu ;

Valencia, Julio C. ;

Sanford, Michael ;

Fenimore, John M. ;

Rahman, Ziaur S. M. ;

Tsuneyama, Koichi ;

Norman, Gary L. ;

Gershwin, M. Eric ;

Young, Howard A. .

HEPATOLOGY, 2018, 67 (04) :1408-1419

[3] Chronic Expression of Interferon-Gamma Leads to Murine Autoimmune Cholangitis With a Female Predominance [J].

Bae, Heekyong R. ;

Leung, Patrick S. C. ;

Tsuneyama, Koichi ;

Valencia, Julio C. ;

Hodge, Deborah L. ;

Kim, Seohyun ;

Back, Tim ;

Karwan, Megan ;

Merchant, Anand S. ;

Baba, Nobuyuki ;

Feng, Dechun ;

Park, Ogyi ;

Gao, Bin ;

Yang, Guo-Xiang ;

Gershwin, M. Eric ;

Young, Howard A. .

HEPATOLOGY, 2016, 64 (04) :1189-1201

[4] The challenges of primary biliary cholangitis: What is new and what needs to be done [J].

Benedetta, Terziroli Beretta-Piccoli ;

Mieli-Vergani, Giorgina ;

Vergani, Diego ;

Vierling, John M. ;

Adams, David ;

Alpini, Gianfranco ;

Banales, Jesus M. ;

Beuers, Ulrich ;

Bjornsson, Einar ;

Bowlus, Christopher ;

Carbone, Marco ;

Chazouilleres, Olivier ;

Dalekos, George ;

De Gottardi, Andrea ;

Harada, Kenichi ;

Hirschfield, Gideon ;

Invernizzi, Pietro ;

Jones, David ;

Krawitt, Edward ;

Lanzavecchia, Antonio ;

Lian, Zhe-Xiong ;

Ma, Xiong ;

Manns, Michael ;

Mavilio, Domenico ;

Quigley, Eamon M. M. ;

Sallusto, Federica ;

Shimoda, Shinji ;

Strazzabosco, Mario ;

Swain, Mark ;

Tanaka, Atsushi ;

Trauner, Michael ;

Tsuneyama, Koichi ;

Zigmond, Ehud ;

Gershwin, M. Eric .

JOURNAL OF AUTOIMMUNITY, 2019, 105

[5] Interleukin-30 Suppresses Not Only CD4+ T Cells but Also Regulatory T Cells in Murine Primary Biliary Cholangitis [J].

Chen, Hung-Wen ;

Lin, Chia-, I ;

Chuang, Ya-Hui .

BIOMEDICINES, 2021, 9 (08)

[6] CXCL12-CXCR4-Mediated Chemotaxis Supports Accumulation of Mucosal-Associated Invariant T Cells Into the Liver of Patients With PBC [J].

Chen, Zhilei ;

Liu, Suying ;

He, Chengmei ;

Sun, Jinlei ;

Wang, Li ;

Chen, Hua ;

Zhang, Fengchun .

FRONTIERS IN IMMUNOLOGY, 2021, 12

[7] CD8+ T cells undergo activation and programmed death-1 repression in the liver of aged Ae2a,b-/- mice favoring autoimmune cholangitis [J].

Concepcion, Axel R. ;

Salas, January T. ;

Saez, Elena ;

Sarvide, Sarai ;

Ferrer, Alex ;

Portu, Ainhoa ;

Uriarte, Iker ;

Hervas-Stubbs, Sandra ;

Elferink, Ronald P. J. Oude ;

Prieto, Jesus ;

Medina, Juan F. .

ONCOTARGET, 2015, 6 (30) :28588-28606

[8] Anion exchanger 2 is critical for CD8+T cells to maintain pHi homeostasis and modulate immune responses [J].

Concepcion, Axel R. ;

Salas, January T. ;

Sarvide, Sarai ;

Saez, Elena ;

Ferrer, Alex ;

Lopez, Maria ;

Portu, Ainhoa ;

Banales, Jesus M. ;

Hervas-Stubbs, Sandra ;

Elferink, Ronald P. J. Oude ;

Prieto, Jesus ;

Medina, Juan F. .

EUROPEAN JOURNAL OF IMMUNOLOGY, 2014, 44 (05) :1341-1351

[9] Demographic, lifestyle, medical and familial factors associated with primary biliary cirrhosis [J].

Corpechot, Christophe ;

Chretien, Yves ;

Chazouilleres, Olivier ;

Poupon, Raoul .

JOURNAL OF HEPATOLOGY, 2010, 53 (01) :162-169

[10] B Cell Depletion Therapy Exacerbates Murine Primary Biliary Cirrhosis [J].

Dhirapong, Amy ;

Lleo, Ana ;

Yang, Guo-Xiang ;

Tsuneyama, Koichi ;

Dunn, Robert ;

Kehry, Marilyn ;

Packard, Thomas A. ;

Cambier, John C. ;

Liu, Fu-Tong ;

Lindor, Keith ;

Coppel, Ross L. ;

Ansari, Aftab A. ;

Gershwin, M. Eric .

HEPATOLOGY, 2011, 53 (02) :527-535

← 1 2 3 4 5 6 7 8 9 →