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Overactive WASp in ...
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He, MinghuiKarolinska Institutet
(author)
Overactive WASp in X-linked neutropenia leads to aberrant B-cell division and accelerated plasma cell generation.
- Article/chapterEnglish2022
Publisher, publication year, extent ...
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Elsevier BV,2022
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printrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:kth-352192
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https://urn.kb.se/resolve?urn=urn:nbn:se:kth:diva-352192URI
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https://doi.org/10.1016/j.jaci.2021.07.033DOI
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http://kipublications.ki.se/Default.aspx?queryparsed=id:149067107URI
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Language:English
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Summary in:English
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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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.
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Saeed, MezidaKarolinska Institutet(Swepub:kth)u1omomv1
(author)
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Record, JulienKarolinska Institutet
(author)
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Keszei, MartonKarolinska Institutet
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Gonçalves Pinho, LiaKarolinska Institutet
(author)
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Vasconcelos-Fontes, Larissa
(author)
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D'Aulerio, RobertaKarolinska Institutet
(author)
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Vieira, RhaissaKarolinska Institutet
(author)
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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
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Deordieva, Ekaterina
(author)
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Buedts, Lieselot
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Matias Lopes, Joao Pedro
(author)
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Pershin, Dmitry
(author)
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Hammarström, LennartKarolinska Institutet
(author)
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Xia, Yu
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Zhao, Xiaodong
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Cunningham-Rundles, Charlotte
(author)
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Thrasher, Adrian J
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Burns, Siobhan O
(author)
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Cotta-de-Almeida, Vinicius
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Liu, Chaohong
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Shcherbina, Anna
(author)
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Vandenberghe, Peter
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Westerberg, Lisa SKarolinska Institutet
(author)
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Karolinska Institutet
(creator_code:org_t)
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In:Journal of Allergy and Clinical Immunology: Elsevier BV149:3, s. 1069-10840091-67491097-6825
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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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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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Royal Institute of Technology
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Karolinska Institutet