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He M, Saeed MB, Record J, et al. Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation. J Allergy Clin Immunol. 2021;doi: 10.1016/j.jaci.2021.07.033.
He, M., Saeed, M. B., Record, J., Keszei, M., Gonçalves Pinho, L., Vasconcelos-Fontes, L., D'Aulerio, R., Vieira, R., Oliveira, M. M. S., Geyer, C., Bohaumilitzky, L., Thiemann, M., Deordieva, E., Buedts, L., Matias Lopes, J. P., Pershin, D., Hammarström, L., Xia, Y., Zhao, X., Cunningham-Rundles, C., Thrasher, A. J., Burns, S. O., Cotta-de-Almeida, V., Liu, C., Shcherbina, A., Vandenberghe, P., & Westerberg, L. S. (2021). Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation. The Journal of allergy and clinical immunology, . https://doi.org/10.1016/j.jaci.2021.07.033
He, Minghui, et al. "Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation." The Journal of allergy and clinical immunology vol. (2021). doi: https://doi.org/10.1016/j.jaci.2021.07.033
He M, Saeed MB, Record J, Keszei M, Gonçalves Pinho L, Vasconcelos-Fontes L, D'Aulerio R, Vieira R, Oliveira MMS, Geyer C, Bohaumilitzky L, Thiemann M, Deordieva E, Buedts L, Matias Lopes JP, Pershin D, Hammarström L, Xia Y, Zhao X, Cunningham-Rundles C, Thrasher AJ, Burns SO, Cotta-de-Almeida V, Liu C, Shcherbina A, Vandenberghe P, Westerberg LS. Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation. J Allergy Clin Immunol. 2021 Aug 09; doi: 10.1016/j.jaci.2021.07.033. Epub 2021 Aug 09. PMID: 34384840.
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J Allergy Clin Immunol. 2021 Aug 09; doi: 10.1016/j.jaci.2021.07.033. Epub 2021 Aug 09.

Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation.

The Journal of allergy and clinical immunology

Minghui He, Mezida B Saeed, Julien Record, Marton Keszei, Lia Gonçalves Pinho, Larissa Vasconcelos-Fontes, Roberta D'Aulerio, Rhaissa Vieira, Mariana M S Oliveira, Chiara Geyer, Lena Bohaumilitzky, Meike Thiemann, Ekaterina Deordieva, Lieselot Buedts, Joao Pedro Matias Lopes, Dmitry Pershin, Lennart Hammarström, Yu Xia, Xiaodong Zhao, Charlotte Cunningham-Rundles, Adrian J Thrasher, Siobhan O Burns, Vinicius Cotta-de-Almeida, Chaohong Liu, Anna Shcherbina, Peter Vandenberghe, Lisa S Westerberg

Affiliations

  1. Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm, Sweden. Electronic address: [email protected].
  2. Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm, Sweden.
  3. Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm, Sweden; Laboratory on Thymus Research, Oswaldo Cruz Institute, Fiocruz, Rio de Janeiro, Brazil.
  4. Department of Immunology, Dmitry Rogachev National Medical and Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
  5. Center for Human Genetics, University Hospital Leuven, Leuven, Belgium.
  6. Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY; Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Cleveland, Ohio.
  7. Department of Laboratory Medicine, Karolinska Institutet, Stockholm, Sweden.
  8. Department of Rheumatology and Immunology, Shenzhen Children's Hospital, Shenzhen, China.
  9. Chongqing Key Laboratory of Child Infection and Immunity, Children's Hospital of Chongqing Medical University, Chongqing, China.
  10. Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY.
  11. UCL Great Ormond Street Institute of Child Health, London, United Kingdom; Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
  12. Department of Immunology, Royal Free London NHS Foundation Trust, London, United Kingdom; Institute of Immunity and Transplantation, University College London, London, United Kingdom.
  13. Laboratory on Thymus Research, Oswaldo Cruz Institute, Fiocruz, Rio de Janeiro, Brazil.
  14. Department of Pathogen Biology, School of Basic Medicine, Huazhong University of Science and Technology, Wuhan, China.
  15. Department of Immunology, Royal Free London NHS Foundation Trust, London, United Kingdom. Electronic address: [email protected].

PMID: 34384840 DOI: 10.1016/j.jaci.2021.07.033

Abstract

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 IgA

CONCLUSIONS: Overactive WASp predisposes B cells for premature differentiation into plasma cells at the expense of cell proliferation and immunoglobulin class switching.

Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

Keywords: B cells; IgA; WASp; X-linked neutropenia; actin; germinal center; plasma cells; primary immunodeficiency

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