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    Science immunology. pii: 5/49/eabc3979. doi: 10.1126/sciimmunol.abc3979
    The cytoskeletal regulator HEM1 governs B cell development and prevents autoimmunity.
    Salzer E1,  Zoghi S2,  Kiss MG3,  Kage F4,  Rashkova C5,  Stahnke S6,  Haimel M7,  Platzer R8,  Caldera M9,  Ardy RC10,  Hoeger B11,  Block J12,  Medgyesi D13,  Sin C14,  Shahkarami S15,  Kain R16,  Ziaee V17,  Hammerl P18,  Bock C19,  Menche J20,  Dupré L21,  Huppa JB22,  Sixt M23,  Lomakin A24,  Rottner K25,  Binder CJ26,  Stradal TEB27,  Rezaei N28,  Boztug K29
    Author information
    1Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    2Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    3CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
    4Division of Molecular Cell Biology, Zoological Institute, Technische Universität Braunschweig, Braunschweig, Germany.
    5Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    6Department of Cell Biology, Helmholtz Centre for Infection Research, Braunschweig, Germany.
    7Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    8Institute for Hygiene and Applied Immunology, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Vienna, Austria.
    9CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
    10Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    11Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    12Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    13Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    14CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
    15Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
    16Department of Pathology, Medical University of Vienna, Vienna, Austria.
    17Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    18Department of Molecular Biology, University of Salzburg, Salzburg, Austria.
    19Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    20CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
    21Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    22Institute for Hygiene and Applied Immunology, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Vienna, Austria.
    23Institute of Science and Technology Austria, Klosterneuburg, Austria.
    24Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
    25Division of Molecular Cell Biology, Zoological Institute, Technische Universität Braunschweig, Braunschweig, Germany.
    26CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
    27Department of Cell Biology, Helmholtz Centre for Infection Research, Braunschweig, Germany.
    28Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
    29St. Anna Children's Cancer Research Institute (CCRI), Vienna, Austria. kaan.boztug@ccri.at.
    Abstract

    The WAVE regulatory complex (WRC) is crucial for assembly of the peripheral branched actin network constituting one of the main drivers of eukaryotic cell migration. Here, we uncover an essential role of the hematopoietic-specific WRC component HEM1 for immune cell development. Germline-encoded HEM1 deficiency underlies an inborn error of immunity with systemic autoimmunity, at cellular level marked by WRC destabilization, reduced filamentous actin, and failure to assemble lamellipodia. Hem1 mice display systemic autoimmunity, phenocopying the human disease. In the absence of Hem1, B cells become deprived of extracellular stimuli necessary to maintain the strength of B cell receptor signaling at a level permissive for survival of non-autoreactive B cells. This shifts the balance of B cell fate choices toward autoreactive B cells and thus autoimmunity.


    Copyright © 2020 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works.

    Publikations ID: 32646852
    Quelle: öffnen
     
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