期刊
SCIENCE IMMUNOLOGY
卷 5, 期 49, 页码 -出版社
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/sciimmunol.abc3979
关键词
-
类别
资金
- European Research Council Consolidator Grant iDysChart (ERC) [820074]
- FWF [FWF I2250-B28]
- Austrian Society of Pediatrics and Adolescent Medicine
- Deutsche Forschungsgemeinschaft
- Helmholtz Society
- European Research Council (ERC) [820074] Funding Source: European Research Council (ERC)
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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据