---
_id: '8132'
abstract:
- lang: eng
  text: 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.
article_number: eabc3979
article_processing_charge: No
article_type: original
author:
- first_name: Elisabeth
  full_name: Salzer, Elisabeth
  last_name: Salzer
- first_name: Samaneh
  full_name: Zoghi, Samaneh
  last_name: Zoghi
- first_name: Máté G.
  full_name: Kiss, Máté G.
  last_name: Kiss
- first_name: Frieda
  full_name: Kage, Frieda
  last_name: Kage
- first_name: Christina
  full_name: Rashkova, Christina
  last_name: Rashkova
- first_name: Stephanie
  full_name: Stahnke, Stephanie
  last_name: Stahnke
- first_name: Matthias
  full_name: Haimel, Matthias
  last_name: Haimel
- first_name: René
  full_name: Platzer, René
  last_name: Platzer
- first_name: Michael
  full_name: Caldera, Michael
  last_name: Caldera
- first_name: Rico Chandra
  full_name: Ardy, Rico Chandra
  last_name: Ardy
- first_name: Birgit
  full_name: Hoeger, Birgit
  last_name: Hoeger
- first_name: Jana
  full_name: Block, Jana
  last_name: Block
- first_name: David
  full_name: Medgyesi, David
  last_name: Medgyesi
- first_name: Celine
  full_name: Sin, Celine
  last_name: Sin
- first_name: Sepideh
  full_name: Shahkarami, Sepideh
  last_name: Shahkarami
- first_name: Renate
  full_name: Kain, Renate
  last_name: Kain
- first_name: Vahid
  full_name: Ziaee, Vahid
  last_name: Ziaee
- first_name: Peter
  full_name: Hammerl, Peter
  last_name: Hammerl
- first_name: Christoph
  full_name: Bock, Christoph
  last_name: Bock
- first_name: Jörg
  full_name: Menche, Jörg
  last_name: Menche
- first_name: Loïc
  full_name: Dupré, Loïc
  last_name: Dupré
- first_name: Johannes B.
  full_name: Huppa, Johannes B.
  last_name: Huppa
- first_name: Michael K
  full_name: Sixt, Michael K
  id: 41E9FBEA-F248-11E8-B48F-1D18A9856A87
  last_name: Sixt
  orcid: 0000-0002-6620-9179
- first_name: Alexis
  full_name: Lomakin, Alexis
  last_name: Lomakin
- first_name: Klemens
  full_name: Rottner, Klemens
  last_name: Rottner
- first_name: Christoph J.
  full_name: Binder, Christoph J.
  last_name: Binder
- first_name: Theresia E.B.
  full_name: Stradal, Theresia E.B.
  last_name: Stradal
- first_name: Nima
  full_name: Rezaei, Nima
  last_name: Rezaei
- first_name: Kaan
  full_name: Boztug, Kaan
  last_name: Boztug
citation:
  ama: Salzer E, Zoghi S, Kiss MG, et al. The cytoskeletal regulator HEM1 governs
    B cell development and prevents autoimmunity. <i>Science Immunology</i>. 2020;5(49).
    doi:<a href="https://doi.org/10.1126/sciimmunol.abc3979">10.1126/sciimmunol.abc3979</a>
  apa: Salzer, E., Zoghi, S., Kiss, M. G., Kage, F., Rashkova, C., Stahnke, S., …
    Boztug, K. (2020). The cytoskeletal regulator HEM1 governs B cell development
    and prevents autoimmunity. <i>Science Immunology</i>. AAAS. <a href="https://doi.org/10.1126/sciimmunol.abc3979">https://doi.org/10.1126/sciimmunol.abc3979</a>
  chicago: Salzer, Elisabeth, Samaneh Zoghi, Máté G. Kiss, Frieda Kage, Christina
    Rashkova, Stephanie Stahnke, Matthias Haimel, et al. “The Cytoskeletal Regulator
    HEM1 Governs B Cell Development and Prevents Autoimmunity.” <i>Science Immunology</i>.
    AAAS, 2020. <a href="https://doi.org/10.1126/sciimmunol.abc3979">https://doi.org/10.1126/sciimmunol.abc3979</a>.
  ieee: E. Salzer <i>et al.</i>, “The cytoskeletal regulator HEM1 governs B cell development
    and prevents autoimmunity,” <i>Science Immunology</i>, vol. 5, no. 49. AAAS, 2020.
  ista: Salzer E, Zoghi S, Kiss MG, Kage F, Rashkova C, Stahnke S, Haimel M, Platzer
    R, Caldera M, Ardy RC, Hoeger B, Block J, Medgyesi D, Sin C, Shahkarami S, Kain
    R, Ziaee V, Hammerl P, Bock C, Menche J, Dupré L, Huppa JB, Sixt MK, Lomakin A,
    Rottner K, Binder CJ, Stradal TEB, Rezaei N, Boztug K. 2020. The cytoskeletal
    regulator HEM1 governs B cell development and prevents autoimmunity. Science Immunology.
    5(49), eabc3979.
  mla: Salzer, Elisabeth, et al. “The Cytoskeletal Regulator HEM1 Governs B Cell Development
    and Prevents Autoimmunity.” <i>Science Immunology</i>, vol. 5, no. 49, eabc3979,
    AAAS, 2020, doi:<a href="https://doi.org/10.1126/sciimmunol.abc3979">10.1126/sciimmunol.abc3979</a>.
  short: E. Salzer, S. Zoghi, M.G. Kiss, F. Kage, C. Rashkova, S. Stahnke, M. Haimel,
    R. Platzer, M. Caldera, R.C. Ardy, B. Hoeger, J. Block, D. Medgyesi, C. Sin, S.
    Shahkarami, R. Kain, V. Ziaee, P. Hammerl, C. Bock, J. Menche, L. Dupré, J.B.
    Huppa, M.K. Sixt, A. Lomakin, K. Rottner, C.J. Binder, T.E.B. Stradal, N. Rezaei,
    K. Boztug, Science Immunology 5 (2020).
date_created: 2020-07-19T22:00:58Z
date_published: 2020-07-10T00:00:00Z
date_updated: 2023-08-22T07:56:04Z
day: '10'
department:
- _id: MiSi
doi: 10.1126/sciimmunol.abc3979
external_id:
  isi:
  - '000546994600004'
  pmid:
  - '32646852'
intvolume: '         5'
isi: 1
issue: '49'
language:
- iso: eng
month: '07'
oa_version: None
pmid: 1
publication: Science Immunology
publication_identifier:
  eissn:
  - '24709468'
publication_status: published
publisher: AAAS
quality_controlled: '1'
scopus_import: '1'
status: public
title: The cytoskeletal regulator HEM1 governs B cell development and prevents autoimmunity
type: journal_article
user_id: 4359f0d1-fa6c-11eb-b949-802e58b17ae8
volume: 5
year: '2020'
...