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

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

He, Minghui (author): Karolinska Institutet

Saeed, Mezida (author): Karolinska Institutet

Record, Julien (author): Karolinska Institutet

Keszei, Marton (author): Karolinska Institutet

Gonçalves Pinho, Lia (author): Karolinska Institutet

Vasconcelos-Fontes, Larissa (author)

D'Aulerio, Roberta (author): Karolinska Institutet

Vieira, Rhaissa (author): Karolinska Institutet

Oliveira, Mariana M S (author)

Geyer, Chiara (author)

Bohaumilitzky, Lena (author)

Thiemann, Meike (author)

Deordieva, Ekaterina (author)

Buedts, Lieselot (author)

Matias Lopes, Joao Pedro (author)

Pershin, Dmitry (author)

Hammarström, Lennart (author): Karolinska Institutet

Xia, Yu (author)

Zhao, Xiaodong (author)

Cunningham-Rundles, Charlotte (author)

Thrasher, Adrian J (author)

Burns, Siobhan O (author)

Cotta-de-Almeida, Vinicius (author)

Liu, Chaohong (author)

Shcherbina, Anna (author)

Vandenberghe, Peter (author)

Westerberg, Lisa S (author): Karolinska Institutet

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Elsevier BV, 2022
2022
English.
In: Journal of Allergy and Clinical Immunology. - : Elsevier BV. - 0091-6749 .- 1097-6825. ; 149:3, s. 1069-1084

Related links:: http://www.jacionlin...; show more...; https://urn.kb.se/re...; https://doi.org/10.1...; http://kipublication...; show less...

Journal article (peer-reviewed)

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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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MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Basic Medicine; and Immunology in th ...

Articles in the publication: Journal of Aller ...

By the university: Royal Institute of Technology; Karolinska Institutet

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

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