Research advances in animal models of primary biliary cholangitis

Yafei XU; Zhibin ZHAO; Zhexiong LIAN

doi:10.3969/j.issn.1001-5256.2021.10.006

Volume 37 Issue 10

Oct. 2021

Turn off MathJax

Article Contents

Abstract

References

Article Navigation > Journal of Clinical Hepatology > 2021 > 37(10): 2280-2285

PDF( 1944 KB)

Research advances in animal models of primary biliary cholangitis

DOI: 10.3969/j.issn.1001-5256.2021.10.006

1.
School of Medicine, South China University of Technology, Guangzhou 510006, China
2.
Guangdong Provincial People's Hospital, Guangzhou 510000, China

Received Date: 2021-07-20
Accepted Date: 2021-08-25
Published Date: 2021-10-20

Abstract

Abstract

Primary biliary cholangitis (PBC) is an inflammatory and cholestatic liver disease caused by autoimmune response targeting the small- and medium-sized intrahepatic bile ducts. The most specific manifestations of this diseases in clinical practice were positive anti-mitochondrial antibody and selective destruction of small- and medium-sized bile ducts based on liver histology. Since it is difficult to obtain the clinical samples of early-stage PBC, the construction and optimization of mouse models is an important method to investigate the pathogenesis of PBC. An understanding of the modeling principles of PBC animal models and disease features in serology, histology, and cytology not only helps to improve the awareness of PBC, but also helps to design scientific and rational research protocols.
- Autoimmune Diseases,
- Liver Cirrhosis, Biliary,
- Disease Models, Animal

FullText(HTML)

References(35)

References

[1]	SCHEUER PJ. Ludwig Symposium on biliary disorders--part Ⅱ. Pathologic features and evolution of primary biliary cirrhosis and primary sclerosing cholangitis[J]. Mayo Clin Proc, 1998, 73(2): 179-183. DOI: 10.4065/73.2.179.
[2]	VLEGGAAR FP, van BUUREN HR. No prognostic significance of antimitochondrial antibody profile testing in primary biliary cirrhosis[J]. Hepatogastroenterology, 2004, 51(58): 937-940.
[3]	GORELIK L, FLAVELL RA. Abrogation of TGFbeta signaling in T cells leads to spontaneous T cell differentiation and autoimmune disease[J]. Immunity, 2000, 12(2): 171-181. DOI: 10.1016/s1074-7613(00)80170-3.
[4]	HUANG MX, YANG SY, LUO PY, et al. Gut microbiota contributes to sexual dimorphism in murine autoimmune cholangitis[J]. J Leukoc Biol, 2021. DOI: 10.1002/JLB.3MA0321-037R.[Online abead of print]
[5]	NAKAMURA A, YAMAZAKI K, SUZUKI K, et al. Increased portal tract infiltration of mast cells and eosinophils in primary biliary cirrhosis[J]. Am J Gastroenterol, 1997, 92(12): 2245-2249.
[6]	HODGE DL, BERTHET C, COPPOLA V, et al. IFN-gamma AU-rich element removal promotes chronic IFN-gamma expression and autoimmunity in mice[J]. J Autoimmun, 2014, 53: 33-45. DOI: 10.1016/j.jaut.2014.02.003.
[7]	BAE HR, LEUNG PS, TSUNEYAMA K, et al. Chronic expression of interferon-gamma leads to murine autoimmune cholangitis with a female predominance[J]. Hepatology, 2016, 64(4): 1189-1201. DOI: 10.1002/hep.28641.
[8]	IRIE J, WU Y, WICKER LS, et al. NOD. c3c4 congenic mice develop autoimmune biliary disease that serologically and pathogenetically models human primary biliary cirrhosis[J]. J Exp Med, 2006, 203(5): 1209-1219. DOI: 10.1084/jem.20051911.
[9]	ZHANG W, SHARMA R, JU ST, et al. Deficiency in regulatory T cells results in development of antimitochondrial antibodies and autoimmune cholangitis[J]. Hepatology, 2009, 49(2): 545-552. DOI: 10.1002/hep.22651.
[10]	GODFREY VL, WILKINSON JE, RUSSELL LB. X-linked lymphoreticular disease in the scurfy (sf) mutant mouse[J]. Am J Pathol, 1991, 138(6): 1379-1387.
[11]	NELSON BH. IL-2, regulatory T cells, and tolerance[J]. J Immunol, 2004, 172(7): 3983-3988. DOI: 10.4049/jimmunol.172.7.3983.
[12]	WAKABAYASHI K, LIAN ZX, MORITOKI Y, et al. IL-2 receptor alpha(-/-) mice and the development of primary biliary cirrhosis[J]. Hepatology, 2006, 44(5): 1240-1249. DOI: 10.1002/hep.21385.
[13]	HSU W, ZHANG W, TSUNEYAMA K, et al. Differential mechanisms in the pathogenesis of autoimmune cholangitis versus inflammatory bowel disease in interleukin-2Ralpha(-/-) mice[J]. Hepatology, 2009, 49(1): 133-140. DOI: 10.1002/hep.22591.
[14]	ZHU J, YAMANE H, PAUL WE. Differentiation of effector CD4 T cell populations (*)[J]. Annu Rev Immunol, 2010, 28: 445-489. DOI: 10.1146/annurev-immunol-030409-101212.
[15]	YAO Y, YANG W, YANG YQ, et al. Distinct from its canonical effects, deletion of IL-12p40 induces cholangitis and fibrosis in interleukin-2Rα(-/-) mice[J]. J Autoimmun, 2014, 51: 99-108. DOI: 10.1016/j.jaut.2014.02.009.
[16]	LINDOR KD, GERSHWIN ME, POUPON R, et al. Primary biliary cirrhosis[J]. Hepatology (Baltimore, Md), 2009, 50(1): 291-308. DOI: 10.1002/hep.22906.
[17]	GAO CY, YAO Y, LI L, et al. Tissue-resident memory CD8⁺ T cells acting as mediators of salivary gland damage in a murine model of sjögren's syndrome[J]. Arthritis Rheumatol, 2019, 71(1): 121-132. DOI: 10.1002/art.40676.
[18]	SUN Y, ZHANG W, LI B, et al. The coexistence of Sjögren's syndrome and primary biliary cirrhosis: A comprehensive review[J]. Clin Rev Allergy Immunol, 2015, 48(2-3): 301-315. DOI: 10.1007/s12016-015-8471-1.
[19]	YAO Y, LI L, YANG SH, et al. CD8⁺ T cells and IFN-γ induce autoimmune myelofibrosis in mice[J]. J Autoimmun, 2018, 89: 101-111. DOI: 10.1016/j.jaut.2017.12.011.
[20]	ROMERO MF, FULTON CM, BORON WF. The SLC₄ family of HCO₃^- transporters[J]. Pflugers Arch, 2004, 447(5): 495-509. DOI: 10.1007/s00424-003-1180-2.
[21]	GAWENIS LR, LEDOUSSAL C, JUDD LM, et al. Mice with a targeted disruption of the AE2 Cl^-/HCO3^- exchanger are achlorhydric[J]. J Biol Chem, 2004, 279(29): 30531-30539. DOI: 10.1074/jbc.M403779200.
[22]	BANALES JM, ARENAS F, RODRÍGUEZ-ORTIGOSA CM, et al. Bicarbonate-rich choleresis induced by secretin in normal rat is taurocholate-dependent and involves AE2 anion exchanger[J]. Hepatology, 2006, 43(2): 266-275. DOI: 10.1002/hep.21042.
[23]	SALAS JT, BANALES JM, SARVIDE S, et al. Ae2a, b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis[J]. Gastroenterology, 2008, 134(5): 1482-1493. DOI: 10.1053/j.gastro.2008.02.020.
[24]	RIEGER R, GERSHWIN ME. The X and why of xenobiotics in primary biliary cirrhosis[J]. J Autoimmun, 2007, 28(2-3): 76-84. DOI: 10.1016/j.jaut.2007.02.003.
[25]	RIEGER R, LEUNG PS, JEDDELOH MR, et al. Identification of 2-nonynoic acid, a cosmetic component, as a potential trigger of primary biliary cirrhosis[J]. J Autoimmun, 2006, 27(1): 7-16. DOI: 10.1016/j.jaut.2006.06.002.
[26]	AMANO K, LEUNG PS, RIEGER R, et al. Chemical xenobiotics and mitochondrial autoantigens in primary biliary cirrhosis: Identification of antibodies against a common environmental, cosmetic, and food additive, 2-octynoic acid[J]. J Immunol, 2005, 174(9): 5874-5883. DOI: 10.4049/jimmunol.174.9.5874.
[27]	OPDYKE DL. Monographs on fragrance raw materials[J]. Food Cosmet Toxicol, 1979, 17(4): 357-390. DOI: 10.1016/0015-6264(79)90330-4.
[28]	WAKABAYASHI K, LIAN ZX, LEUNG PS, et al. Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease[J]. Hepatology, 2008, 48(2): 531-540. DOI: 10.1002/hep.22390.
[29]	WAKABAYASHI K, YOSHIDA K, LEUNG PS, et al. Induction of autoimmune cholangitis in non-obese diabetic (NOD). 1101 mice following a chemical xenobiotic immunization[J]. Clin Exp Immunol, 2009, 155(3): 577-586. DOI: 10.1111/j.1365-2249.2008.03837.x.
[30]	ALEXOPOULOU L, HOLT AC, MEDZHITOV R, et al. Recognition of double-stranded RNA and activation of NF-kappaB by Toll-like receptor 3[J]. Nature, 2001, 413(6857): 732-738. DOI: 10.1038/35099560.
[31]	OKADA C, AKBAR SM, HORⅡKE N, et al. Early development of primary biliary cirrhosis in female C57BL/6 mice because of poly Ⅰ∶ C administration[J]. Liver Int, 2005, 25(3): 595-603. DOI: 10.1111/j.1478-3231.2005.01043.x.
[32]	JIANG T, HAN Z, CHEN S, et al. Resistance to activation-induced cell death and elevated FLIPL expression of CD4⁺ T cells in a polyI∶ C-induced primary biliary cirrhosis mouse model[J]. Clin Exp Med, 2009, 9(4): 269-276. DOI: 10.1007/s10238-009-0052-2.
[33]	AMBROSINI YM, YANG GX, ZHANG W, et al. The multi-hit hypothesis of primary biliary cirrhosis: Polyinosinic-polycytidylic acid (poly Ⅰ∶ C) and murine autoimmune cholangitis[J]. Clin Exp Immunol, 2011, 166(1): 110-120. DOI: 10.1111/j.1365-2249.2011.04453.x.
[34]	GERSHWIN ME, SELMI C, WORMAN HJ, et al. Risk factors and comorbidities in primary biliary cirrhosis: A controlled interview-based study of 1032 patients[J]. Hepatology, 2005, 42(5): 1194-1202. DOI: 10.1002/hep.20907.
[35]	HIRSCHFIELD GM, DYSON JK, ALEXANDER G, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines[J]. Gut, 2018, 67(9): 1568-1594. DOI: 10.1136/gutjnl-2017-315259.

Relative Articles

[1]	Xiaoqiang GAO, Shi ZUO, Xiaodong JIA, Yinying LU. Research advances in sodium taurocholate cotransporting polypeptide in hepatobiliary diseases[J]. Journal of Clinical Hepatology, 2022, 38(5): 1179-1182. doi: 10.3969/j.issn.1001-5256.2022.05.043
[2]	Chang LI, Lei YAN, Li WANG, Chongxu HAN, Yunfeng YE, Defu JIN, Yuzhang JIANG. Value of serum IgG4 level in differential diagnosis of IgG4-related pancreatic and hepatobiliary diseases and non-IgG4-related pancreatic and hepatobiliary diseases[J]. Journal of Clinical Hepatology, 2022, 38(6): 1307-1310. doi: 10.3969/j.issn.1001-5256.2022.06.017
[3]	Yulong YANG, Taohua WEI, Wenming YANG, Wenjie HAO, Yue YANG, Nannan QIAN, Xiang LI, Hailin JIANG. Research advances in animal models of Wilson's disease[J]. Journal of Clinical Hepatology, 2022, 38(5): 1169-1174. doi: 10.3969/j.issn.1001-5256.2022.05.041
[4]	Mengqiang CAI, Sutong LIU, Junying LIU, Lihui ZHANG, Wenxia ZHAO. The role of microRNA in autoimmune liver diseases[J]. Journal of Clinical Hepatology, 2021, 37(1): 212-215. doi: 10.3969/j.issn.1001-5256.2021.01.047
[5]	Zhan WANG, Lin WANG, Yuan YAO, Jing LIU, Guirong SUN, Mingjun LIU. Value and clinical application of multiplex bead-based flow fluorescent immunoassay in the detection of autoantibodies in primary biliary cholangitis[J]. Journal of Clinical Hepatology, 2021, 37(10): 2384-2388. doi: 10.3969/j.issn.1001-5256.2021.10.025
[6]	Mingli HU, Qixia WANG, Xiong MA. Considerations on the elevation of aminotransferases in primary biliary cholangitis[J]. Journal of Clinical Hepatology, 2021, 37(10): 2277-2279. doi: 10.3969/j.issn.1001-5256.2021.10.005
[7]	Qiufeng XIAO, Yunyun FEI. The relationship between primary biliary cholangitis and Sjögren's syndrome[J]. Journal of Clinical Hepatology, 2021, 37(10): 2262-2268. doi: 10.3969/j.issn.1001-5256.2021.10.002
[8]	Meng XIA, Yuhao SUN, Meng WANG, Jing LENG. Research advances in commonly used animal models of primary hepatocellular carcinoma[J]. Journal of Clinical Hepatology, 2021, 37(8): 1938-1942. doi: 10.3969/j.issn.1001-5256.2021.08.042
[9]	Lili ZHANG, Aiwen GENG, Chuanwang QI, Jianchun XIAN. Association between immunoglobulin G4-related diseases and autoimmune hepatitis[J]. Journal of Clinical Hepatology, 2021, 37(12): 2972-2975. doi: 10.3969/j.issn.1001-5256.2021.12.050
[10]	Yu LeLe, Xiong Fang, Lu Jun. IgG4-related biliary and pancreatic diseases with significantly elevated CA19-9: A case report[J]. Journal of Clinical Hepatology, 2020, 36(5): 1129-1130. doi: 10.3969/j.issn.1001-5256.2020.05.039
[11]	Tu RongFang, Yang Xue, Tang YingMei. Research advances in primary biliary cholangitis with extrahepatic autoimmune diseases[J]. Journal of Clinical Hepatology, 2020, 36(6): 1398-1401. doi: 10.3969/j.issn.1001-5256.2020.06.046
[12]	Zhang DeHua, Zhao Jie. Pathogenesis of immunoglobulin G4-related hepatobiliary and pancreatic diseases[J]. Journal of Clinical Hepatology, 2019, 35(6): 1401-1405. doi: 10.3969/j.issn.1001-5256.2019.06.050
[13]	Li YanNi, Zhou Lu, Zhang Jie, Deng BaoRu, Guo LiPing, Zhang Jun, Wang BangMao. Chronological changes in clinical features of autoimmune liver disease at initial diagnosis[J]. Journal of Clinical Hepatology, 2017, 33(1): 141-145. doi: 10.3969/j.issn.1001-5256.2017.01.031
[14]	Han Ying, Zhu JiangYi. Autoimmune sclerosing cholangitis: new concept and new evidence[J]. Journal of Clinical Hepatology, 2015, 31(2): 171-173. doi: 10.3969/j.issn.1001-5256.2015.02.006
[15]	Ren Song, Ye ZhaoXiang, Zhu LiMin. Research progress in type I autoimmune pancreatitis[J]. Journal of Clinical Hepatology, 2014, 30(2): 189-193. doi: 10.3969/j.issn.1001-5256.2014.02.023
[16]	Li Ji, Qian JiaMing. Advances in treatment of autoimmune pancreatitis[J]. Journal of Clinical Hepatology, 2013, 29(7): 496-498. doi: 1001-5256 (2013) 07-0496-03
[17]	Bian ZhaoLian, You ZhengRui, Ma Xiong. The clinical analysis on endoscope technique applicated for patients suffering from extrahepatic cholestatic jaundice[J]. Journal of Clinical Hepatology, 2011, 27(6): 601-604.
[18]	Zhou SuMin, Gao PuJun. Advancement in the treatment of two subtypes of autoimmune pancreatitis[J]. Journal of Clinical Hepatology, 2011, 27(11): 1219-1221.
[19]	Zhou SuMin, Gao PuJun, Gao RunPing. A case report of autoimmune pancreatitis[J]. Journal of Clinical Hepatology, 2011, 27(4): 424+43.
[20]	Hu YuLin, Zhang Rong. Clinical features, diagnosis and treatment of autoimmune pancreatitis[J]. Journal of Clinical Hepatology, 2010, 26(5): 487-489.

Supplements(0)

Cited By

Cited by

Periodical cited type(1)

庞浇安，俞渊，胡嗣钦，马天如，叶桂源，常明，李敏鹏，潘孟，陈伟棠. 大黄灵仙方调控IL-2/STAT5信号通路干预Th17/Treg细胞平衡防治肝内胆管炎症. 中药药理与临床. 2025(01): 63-70 .

Other cited types(1)

Proportional views

Proportional views

通讯作者: 陈斌, bchen63@163.com

1.
沈阳化工大学材料科学与工程学院沈阳 110142

Tables(1)

Get Citation

PDF

XML

Article Metrics

Article views (1624) PDF downloads(270)

Research advances in animal models of primary biliary cholangitis

DOI: 10.3969/j.issn.1001-5256.2021.10.006