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Overactive WASp in ...
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation.
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- He, Minghui (author)
- Karolinska Institutet
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- Saeed, Mezida (author)
- Karolinska Institutet
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- Record, Julien (author)
- Karolinska Institutet
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- Keszei, Marton (author)
- Karolinska Institutet
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- Gonçalves Pinho, Lia (author)
- Karolinska Institutet
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Vasconcelos-Fontes, Larissa (author)
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- D'Aulerio, Roberta (author)
- Karolinska Institutet
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- Vieira, Rhaissa (author)
- Karolinska Institutet
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Oliveira, Mariana M S (author)
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Geyer, Chiara (author)
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Bohaumilitzky, Lena (author)
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Thiemann, Meike (author)
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Deordieva, Ekaterina (author)
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Buedts, Lieselot (author)
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Matias Lopes, Joao Pedro (author)
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Pershin, Dmitry (author)
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- Hammarström, Lennart (author)
- Karolinska Institutet
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Xia, Yu (author)
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Zhao, Xiaodong (author)
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Cunningham-Rundles, Charlotte (author)
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Thrasher, Adrian J (author)
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Burns, Siobhan O (author)
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Cotta-de-Almeida, Vinicius (author)
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Liu, Chaohong (author)
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Shcherbina, Anna (author)
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Vandenberghe, Peter (author)
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- Westerberg, Lisa S (author)
- Karolinska Institutet
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(creator_code:org_t)
- Elsevier BV, 2022
- 2022
- English.
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In: Journal of Allergy and Clinical Immunology. - : Elsevier BV. - 0091-6749 .- 1097-6825. ; 149:3, s. 1069-1084
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http://www.jacionlin...
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Subject headings
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- 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+ 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Immunologi inom det medicinska området (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Immunology in the medical area (hsv//eng)
Keyword
- B cells
- IgA
- WASp
- X-linked neutropenia
- actin
- germinal center
- plasma cells
- primary immunodeficiency
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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He, Minghui
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Saeed, Mezida
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Record, Julien
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Keszei, Marton
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Gonçalves Pinho, ...
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Vasconcelos-Font ...
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show more...
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D'Aulerio, Rober ...
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Vieira, Rhaissa
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Oliveira, Marian ...
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Geyer, Chiara
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Bohaumilitzky, L ...
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Thiemann, Meike
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Deordieva, Ekate ...
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Buedts, Lieselot
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Matias Lopes, Jo ...
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Pershin, Dmitry
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Hammarström, Len ...
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Xia, Yu
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Zhao, Xiaodong
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Cunningham-Rundl ...
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Thrasher, Adrian ...
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Burns, Siobhan O
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Cotta-de-Almeida ...
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Liu, Chaohong
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Shcherbina, Anna
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Vandenberghe, Pe ...
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Westerberg, Lisa ...
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Basic Medicine
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and Immunology in th ...
- Articles in the publication
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Journal of Aller ...
- By the university
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Royal Institute of Technology
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Karolinska Institutet