Burbage Marianne, Keppler Selina J, Gasparrini Francesca, Martínez-Martín Nuria, Gaya Mauro, Feest Christoph, Domart Marie-Charlotte, Brakebusch Cord, Collinson Lucy, Bruckbauer Andreas, Batista Facundo D
Lymphocyte Interaction Laboratory, Electron Microscopy Unit, London Research Institute, Cancer Research UK, London WC2A 3LY, England, UK.
Biomedical Institute, Biotech Research and Innovation Centre, University of Copenhagen, 2100 Copenhagen, Denmark.
J Exp Med. 2015 Jan 12;212(1):53-72. doi: 10.1084/jem.20141143. Epub 2014 Dec 29.
The small Rho GTPase Cdc42, known to interact with Wiskott-Aldrich syndrome (WAS) protein, is an important regulator of actin remodeling. Here, we show that genetic ablation of Cdc42 exclusively in the B cell lineage is sufficient to render mice unable to mount antibody responses. Indeed Cdc42-deficient mice are incapable of forming germinal centers or generating plasma B cells upon either viral infection or immunization. Such severe immune deficiency is caused by multiple and profound B cell abnormalities, including early blocks during B cell development; impaired antigen-driven BCR signaling and actin remodeling; defective antigen presentation and in vivo interaction with T cells; and a severe B cell-intrinsic block in plasma cell differentiation. Thus, our study presents a new perspective on Cdc42 as key regulator of B cell physiology.
小Rho GTP酶Cdc42已知可与威斯科特-奥尔德里奇综合征(WAS)蛋白相互作用,是肌动蛋白重塑的重要调节因子。在此,我们表明仅在B细胞谱系中对Cdc42进行基因敲除就足以使小鼠无法产生抗体反应。事实上,Cdc42缺陷小鼠在病毒感染或免疫后无法形成生发中心或产生浆B细胞。这种严重的免疫缺陷是由多种深刻的B细胞异常引起的,包括B细胞发育早期的阻滞;抗原驱动的BCR信号传导和肌动蛋白重塑受损;抗原呈递缺陷以及与T细胞的体内相互作用;以及浆细胞分化中严重的B细胞内在阻滞。因此,我们的研究为Cdc42作为B细胞生理学的关键调节因子提供了新的视角。