Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation
| Autor(a) principal: | |
|---|---|
| Data de Publicação: | 2022 |
| Outros Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , |
| Tipo de documento: | Artigo |
| Idioma: | eng |
| Título da fonte: | Repositório Institucional da FIOCRUZ (ARCA) |
| Texto Completo: | https://www.arca.fiocruz.br/handle/icict/54969 |
Resumo: | Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm. |
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Minghui, HeSaeed, Mezida B.Record, JulienKeszei, MartonPinho, Lia Gonc¸alvesFontes, Larissa VasconcelosD`Aurelio, RobertaVieira, RaissaOliveira, Mariana M. S.Geyer, ChiaraBohaumilitzky, LenaThiemann, MeikeDeordieva, EkaterinaBuedts, LieselotLopes, Joao Pedro MatiasPershin, DmitryHammarström, LennartXia, YuZhao, XiaodongCunningham-Rundles, CharlotteThrasher, Adrian J.Burns, Siobhan O.Almeida, Vinicius Cotta deLiu, ChaohongShcherbina, AnnaVandenberghe, PeterWesterberg, Lisa S.2022-10-03T15:04:04Z2022-10-03T15:04:04Z2022Minghui, He et al. Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation. J. Allergy Clin. Immunol., v. 149, n. 3, p. 1 - 16, Mar. 2022.0091-6749https://www.arca.fiocruz.br/handle/icict/5496910.1016/j.jaci.2021.07.033engElsevierCélulas BNeutropenia ligada ao XActinaVespaIgACentro germinativoCélulas plasmáticasImunodeficiência primáriaB cellsX-linked neutropeniaActinWASpIgAGerminal centerPlasma cellsPrimary immunodeficiencyOveractive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generationinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleDepartment of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm / Fundação Oswaldo Cruz. Laboratório de Pesquisa sobre o Timo. Rio de Janeiro, RJ, Brasil.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm / Fundação Oswaldo Cruz. Laboratório de Pesquisa sobre o Timo. Rio de Janeiro, RJ, Brasil.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm.The Department of Immunology, Dmitry Rogachev National Medical and Research Center of Pediatric Hematology, Oncology and Immunology, MoscowCenter for Human Genetics, University Hospital Leuven, LeuvenDepartment of Pediatrics, Icahn School of Medicine at Mount Sinai, New York / Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, UH Rainbow Babies and Children’s Hospital, Cleveland.Department of Immunology, Dmitry Rogachev National Medical and Research Center of Pediatric Hematology, Oncology and Immunology, Moscow.Department of Laboratory Medicine, Karolinska Institutet, Stockholm.Department of Rheumatology and Immunology, Shenzhen Children’s Hospital, Shenzhen.Chongqing Key Laboratory of Child Infection and Immunity, Children’s Hospital of Chongqing Medical University, Chongqing.Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, USA.UCL Great Ormond Street Institute of Child Health, London / Great Ormond Street Hospital for Children NHS Foundation Trust, London.Chongqing Key Laboratory of Child Infection and Immunity, Children’s Hospital of Chongqing Medical University, Chongqing / Institute of Immunity and Transplantation, University College London, London.Fundação Oswaldo Cruz. Laboratório de Pesquisa sobre o Timo. Rio de Janeiro, RJ, Brasil.Department of Pathogen Biology, School of Basic Medicine, Huazhong University of Science and Technology, Wuhan.Department of Immunology, Dmitry Rogachev National Medical and Research Center of Pediatric Hematology, Oncology and Immunology, Moscow.Center for Human Genetics, University Hospital Leuven, Leuven.Chongqing Key Laboratory of Child Infection and Immunity, Children’s Hospital of Chongqing Medical University, Chongqing.Background: B-cell affinity maturation in germinal center relies on regulated actin dynamics for cell migration and cell-to-cell communication. Activating mutations in the cytoskeletal regulator Wiskott-Aldrich syndrome protein (WASp) cause X-linked neutropenia (XLN) with reduced serum level of IgA. Objective: We investigated the role of B cells in XLN pathogenesis. Methods: We examined B cells from 6 XLN patients, 2 of whom had novel R268W and S271F mutations in WASp. By using immunized XLN mouse models that carry the corresponding patient mutations, WASp L272P or WASp I296T, we examined the B-cell response. Results: XLN patients had normal naive B cells and plasmablasts, but reduced IgA1 B cells and memory B cells, and poor B-cell proliferation. On immunization, XLN mice had a 2-fold reduction in germinal center B cells in spleen, but with increased generation of plasmablasts and plasma cells. In vitro, XLN B cells showed reduced immunoglobulin class switching and aberrant cell division as well as increased production of immunoglobulin-switched plasma cells. Conclusions: Overactive WASp predisposes B cells for premature differentiation into plasma cells at the expense of cell proliferation and immunoglobulin class switching. 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| dc.title.en_US.fl_str_mv |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation |
| title |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation |
| spellingShingle |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation Minghui, He Células B Neutropenia ligada ao X Actina Vespa IgA Centro germinativo Células plasmáticas Imunodeficiência primária B cells X-linked neutropenia Actin WASp IgA Germinal center Plasma cells Primary immunodeficiency |
| title_short |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation |
| title_full |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation |
| title_fullStr |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation |
| title_full_unstemmed |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation |
| title_sort |
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation |
| author |
Minghui, He |
| author_facet |
Minghui, He Saeed, Mezida B. Record, Julien Keszei, Marton Pinho, Lia Gonc¸alves Fontes, Larissa Vasconcelos D`Aurelio, Roberta Vieira, Raissa Oliveira, Mariana M. S. Geyer, Chiara Bohaumilitzky, Lena Thiemann, Meike Deordieva, Ekaterina Buedts, Lieselot Lopes, Joao Pedro Matias Pershin, Dmitry Hammarström, Lennart Xia, Yu Zhao, Xiaodong Cunningham-Rundles, Charlotte Thrasher, Adrian J. Burns, Siobhan O. Almeida, Vinicius Cotta de Liu, Chaohong Shcherbina, Anna Vandenberghe, Peter Westerberg, Lisa S. |
| author_role |
author |
| author2 |
Saeed, Mezida B. Record, Julien Keszei, Marton Pinho, Lia Gonc¸alves Fontes, Larissa Vasconcelos D`Aurelio, Roberta Vieira, Raissa Oliveira, Mariana M. S. Geyer, Chiara Bohaumilitzky, Lena Thiemann, Meike Deordieva, Ekaterina Buedts, Lieselot Lopes, Joao Pedro Matias Pershin, Dmitry Hammarström, Lennart Xia, Yu Zhao, Xiaodong Cunningham-Rundles, Charlotte Thrasher, Adrian J. Burns, Siobhan O. Almeida, Vinicius Cotta de Liu, Chaohong Shcherbina, Anna Vandenberghe, Peter Westerberg, Lisa S. |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.contributor.author.fl_str_mv |
Minghui, He Saeed, Mezida B. Record, Julien Keszei, Marton Pinho, Lia Gonc¸alves Fontes, Larissa Vasconcelos D`Aurelio, Roberta Vieira, Raissa Oliveira, Mariana M. S. Geyer, Chiara Bohaumilitzky, Lena Thiemann, Meike Deordieva, Ekaterina Buedts, Lieselot Lopes, Joao Pedro Matias Pershin, Dmitry Hammarström, Lennart Xia, Yu Zhao, Xiaodong Cunningham-Rundles, Charlotte Thrasher, Adrian J. Burns, Siobhan O. Almeida, Vinicius Cotta de Liu, Chaohong Shcherbina, Anna Vandenberghe, Peter Westerberg, Lisa S. |
| dc.subject.other.en_US.fl_str_mv |
Células B Neutropenia ligada ao X Actina Vespa IgA Centro germinativo Células plasmáticas Imunodeficiência primária |
| topic |
Células B Neutropenia ligada ao X Actina Vespa IgA Centro germinativo Células plasmáticas Imunodeficiência primária B cells X-linked neutropenia Actin WASp IgA Germinal center Plasma cells Primary immunodeficiency |
| dc.subject.en.en_US.fl_str_mv |
B cells X-linked neutropenia Actin WASp IgA Germinal center Plasma cells Primary immunodeficiency |
| description |
Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm. |
| publishDate |
2022 |
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2022-10-03T15:04:04Z |
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2022-10-03T15:04:04Z |
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2022 |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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Minghui, He et al. Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation. J. Allergy Clin. Immunol., v. 149, n. 3, p. 1 - 16, Mar. 2022. |
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https://www.arca.fiocruz.br/handle/icict/54969 |
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0091-6749 |
| dc.identifier.doi.none.fl_str_mv |
10.1016/j.jaci.2021.07.033 |
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Minghui, He et al. Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation. J. Allergy Clin. Immunol., v. 149, n. 3, p. 1 - 16, Mar. 2022. 0091-6749 10.1016/j.jaci.2021.07.033 |
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Elsevier |
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Elsevier |
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