Liver Immune Profiling Reveals Pathogenesis and Therapeutics for Biliary Atresia
Biliary atresia (BA) is a severe cholangiopathy that leads to liver failure in infants, but its pathogenesis remains to be fully characterized. By single-cell RNA profiling, we observed macrophage hypo-inflammation, Kupffer cell scavenger function defects, cytotoxic T cell expansion, and deficiency...
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23.12.2020
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| Abstract | Biliary atresia (BA) is a severe cholangiopathy that leads to liver failure in infants, but its pathogenesis remains to be fully characterized. By single-cell RNA profiling, we observed macrophage hypo-inflammation, Kupffer cell scavenger function defects, cytotoxic T cell expansion, and deficiency of CX3CR1
effector T and natural killer (NK) cells in infants with BA. More importantly, we discovered that hepatic B cell lymphopoiesis did not cease after birth and that tolerance defects contributed to immunoglobulin G (IgG)-autoantibody accumulation in BA. In a rhesus-rotavirus induced BA model, depleting B cells or blocking antigen presentation ameliorated liver damage. In a pilot clinical study, we demonstrated that rituximab was effective in depleting hepatic B cells and restoring the functions of macrophages, Kupffer cells, and T cells to levels comparable to those of control subjects. In summary, our comprehensive immune profiling in infants with BA had educed that B-cell-modifying therapies may alleviate liver pathology. |
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| AbstractList | Biliary atresia (BA) is a severe cholangiopathy that leads to liver failure in infants, but its pathogenesis remains to be fully characterized. By single-cell RNA profiling, we observed macrophage hypo-inflammation, Kupffer cell scavenger function defects, cytotoxic T cell expansion, and deficiency of CX3CR1
effector T and natural killer (NK) cells in infants with BA. More importantly, we discovered that hepatic B cell lymphopoiesis did not cease after birth and that tolerance defects contributed to immunoglobulin G (IgG)-autoantibody accumulation in BA. In a rhesus-rotavirus induced BA model, depleting B cells or blocking antigen presentation ameliorated liver damage. In a pilot clinical study, we demonstrated that rituximab was effective in depleting hepatic B cells and restoring the functions of macrophages, Kupffer cells, and T cells to levels comparable to those of control subjects. In summary, our comprehensive immune profiling in infants with BA had educed that B-cell-modifying therapies may alleviate liver pathology. Biliary atresia (BA) is a severe cholangiopathy that leads to liver failure in infants, but its pathogenesis remains to be fully characterized. By single-cell RNA profiling, we observed macrophage hypo-inflammation, Kupffer cell scavenger function defects, cytotoxic T cell expansion, and deficiency of CX3CR1+effector T and natural killer (NK) cells in infants with BA. More importantly, we discovered that hepatic B cell lymphopoiesis did not cease after birth and that tolerance defects contributed to immunoglobulin G (IgG)-autoantibody accumulation in BA. In a rhesus-rotavirus induced BA model, depleting B cells or blocking antigen presentation ameliorated liver damage. In a pilot clinical study, we demonstrated that rituximab was effective in depleting hepatic B cells and restoring the functions of macrophages, Kupffer cells, and T cells to levels comparable to those of control subjects. In summary, our comprehensive immune profiling in infants with BA had educed that B-cell-modifying therapies may alleviate liver pathology.Biliary atresia (BA) is a severe cholangiopathy that leads to liver failure in infants, but its pathogenesis remains to be fully characterized. By single-cell RNA profiling, we observed macrophage hypo-inflammation, Kupffer cell scavenger function defects, cytotoxic T cell expansion, and deficiency of CX3CR1+effector T and natural killer (NK) cells in infants with BA. More importantly, we discovered that hepatic B cell lymphopoiesis did not cease after birth and that tolerance defects contributed to immunoglobulin G (IgG)-autoantibody accumulation in BA. In a rhesus-rotavirus induced BA model, depleting B cells or blocking antigen presentation ameliorated liver damage. In a pilot clinical study, we demonstrated that rituximab was effective in depleting hepatic B cells and restoring the functions of macrophages, Kupffer cells, and T cells to levels comparable to those of control subjects. In summary, our comprehensive immune profiling in infants with BA had educed that B-cell-modifying therapies may alleviate liver pathology. |
| Author | Lew, Andrew M Lin, Yuzhen Huang, Bing Wang, Xiaohui Zeng, Liang Chen, Zhanghua Wu, Qiang Wu, Qi Lin, Zefeng Lu, Bingtai Gu, Xiaoqiong Zhang, Tong Zhang, Yan Zhao, Shanmeizi Delfouneso, David Tong, Yanlu Liang, Jiankun Guo, Xuanjie Zhang, Li Bai, Fan Lu, Liwei Wang, Jun Zhang, Meixue Xu, Yanhui Xu, Yiping Wen, Zhe Zhang, Yuxia Zeng, Jixiao Nutt, Stephen L Liang, Huiying Chen, Zhuang-Gui Yi, Shuhong Xian, Huifang Chai, Chengwei Nie, Junli Xia, Huimin |
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Biomedical Pioneering Innovation Center, Beijing Advanced Innovation Center for Genomics (IGS), School of Life Sciences, Peking University, Beijing, 100871, China – sequence: 4 givenname: Jiankun surname: Liang fullname: Liang, Jiankun organization: Department of Pediatric Surgery, Guangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangdong Provincial Children's Medical Research Center, Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, State Key Laboratory of Respiratory Diseases, Guangzhou Medical University, Guangzhou, 510623, Guangdong, China – sequence: 5 givenname: Zefeng surname: Lin fullname: Lin, Zefeng organization: Department of Pediatric Surgery, Guangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangdong Provincial Children's Medical Research Center, Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, State Key Laboratory of Respiratory Diseases, 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surname: Zhang fullname: Zhang, Tong organization: Department of Pediatrics and Hepatic Surgery, Liver Transplant Center, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, 510630, China – sequence: 28 givenname: David surname: Delfouneso fullname: Delfouneso, David organization: Department of Pediatric Surgery, Guangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangdong Provincial Children's Medical Research Center, Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, State Key Laboratory of Respiratory Diseases, Guangzhou Medical University, Guangzhou, 510623, Guangdong, China – sequence: 29 givenname: Yan surname: Zhang fullname: Zhang, Yan organization: Department of Pediatric Surgery, Guangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangdong Provincial Children's Medical Research Center, Guangzhou Institute of Pediatrics, Guangzhou Women and Children's 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Beijing Advanced Innovation Center for Genomics (IGS), School of Life Sciences, Peking University, Beijing, 100871, China; Center for Translational Cancer Research, First Hospital, Peking University, Beijing 100871, China. 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Electronic address: wenzhe2005@163.com – sequence: 36 givenname: Yuxia surname: Zhang fullname: Zhang, Yuxia email: yuxia.zhang@gwcmc.org organization: Department of Pediatric Surgery, Guangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangdong Provincial Children's Medical Research Center, Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, State Key Laboratory of Respiratory Diseases, Guangzhou Medical University, Guangzhou, 510623, Guangdong, China; The Third Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, China. Electronic address: yuxia.zhang@gwcmc.org |
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| Keywords | CX3CR1 B cell haematopoiesis biliary atresia scRNA-seq autoantibody hypo-inflammation Rituximab cytotoxicity TNFSF13B |
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| References | 33510462 - Nat Rev Gastroenterol Hepatol. 2021 Apr;18(4):219-220 35590128 - Med (N Y). 2021 Jan 15;2(1):14-15 |
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| SubjectTerms | Animals Antigens, CD20 - metabolism B-Lymphocytes - immunology Biliary Atresia - blood Biliary Atresia - drug therapy Biliary Atresia - immunology Biliary Atresia - therapy Biopsy Cell Death Cell Line Cell Proliferation Cell Transdifferentiation Child Child, Preschool Cohort Studies CX3C Chemokine Receptor 1 - metabolism Cytotoxicity, Immunologic Disease Models, Animal Female Humans Immunoglobulin G - metabolism Infant Inflammation - pathology Killer Cells, Natural - immunology Kupffer Cells - pathology Liver - immunology Liver - pathology Liver Cirrhosis - blood Liver Cirrhosis - complications Liver Cirrhosis - immunology Liver Cirrhosis - pathology Lymphocyte Depletion Lymphopoiesis Male Mice, Inbred BALB C Phagocytosis Rituximab - administration & dosage Rituximab - pharmacology Rituximab - therapeutic use RNA - metabolism Rotavirus - physiology Single-Cell Analysis Th1 Cells - immunology Th17 Cells - immunology |
| Title | Liver Immune Profiling Reveals Pathogenesis and Therapeutics for Biliary Atresia |
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| Volume | 183 |
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