Dataset Information

Loss of runx1 function results in B cell immunodeficiency but not T cell in adult zebrafish.

ABSTRACT: Transcription factor RUNX1 holds an integral role in multiple-lineage haematopoiesis and is implicated as a cofactor in V(D)J rearrangements during lymphocyte development. Runx1 deficiencies resulted in immaturity and reduction of lymphocytes in mice. In this study, we found that runx1W84X/W84X mutation led to the reduction and disordering of B cells, as well as the failure of V(D)J rearrangements in B cells but not T cells, resulting in antibody-inadequate-mediated immunodeficiency in adult zebrafish. By contrast, T cell development was not affected. The decreased number of B cells mainly results from excessive apoptosis in immature B cells. Disrupted B cell development results in runx1W84X/W84X mutants displaying a similar phenotype to common variable immunodeficiency-a primary immunodeficiency disease primarily characterized by frequent susceptibility to infection and deficient immune response, with marked reduction of antibody production of IgG, IgA and/or IgM. Our studies demonstrated an evolutionarily conserved function of runx1 in maturation and differentiation of B cells in adult zebrafish, which will serve as a valuable model for the study of immune deficiency diseases and their treatments.

SUBMITTER: Chi Y

PROVIDER: S-EPMC6070721 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature

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Loss of runx1 function results in B cell immunodeficiency but not T cell in adult zebrafish.

Chi Yali Y Huang Zhibin Z Chen Qi Q Xiong Xiaojie X Chen Kemin K Xu Jin J Zhang Yiyue Y Zhang Wenqing W

Open biology 20180701 7

Transcription factor RUNX1 holds an integral role in multiple-lineage haematopoiesis and is implicated as a cofactor in V(D)J rearrangements during lymphocyte development. Runx1 deficiencies resulted in immaturity and reduction of lymphocytes in mice. In this study, we found that runx1W84X/W84X mutation led to the reduction and disordering of B cells, as well as the failure of V(D)J rearrangements in B cells but not T cells, resulting in antibody-inadequate-mediated immu ...[more]

PMID: 30045885

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Dataset Information

Loss of runx1 function results in B cell immunodeficiency but not T cell in adult zebrafish.

Publications

Loss of <i>runx1</i> function results in B cell immunodeficiency but not T cell in adult zebrafish.

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