Autoimmunity because of defective B cell fate decisions in autoimmune lymphoproliferative syndrome (B02)

该项目将继续研究ALPS中有缺陷的非凋亡FAS信号与自身抗体介导的自身免疫之间的联系。特别是，B02旨在揭示B细胞发育紊乱、滤泡外反应增强和自身抗体产生之间的联系。由于干扰信号是该观察的基础，该项目将探索如何通过FAS表达或信号调节B细胞受体激活，以及这如何有助于B细胞的发育决策和在ALPS中观察到的免疫病理。最后，B02将探讨fas触发的B细胞在淋巴组织中的定位和细胞间相互作用。

This project will continue working on the link between defective non-apoptotic FAS signaling and autoantibody mediated autoimmunity in ALPS. In particular, B02 intends to reveal the link between the disturbed development of B cells, enhanced extrafollicular response and the generation of autoantibodies. As disturbed signaling is at the base of this observation, the project will explore how B cell receptor activation is modulated by FAS expression or signaling and how this contributes to developmental decisions in B cells and to the immunopathology observed in ALPS. Finally, B02 will explore the localization and cell-cell interaction of Fas-triggered B cells in lymphoid tissues.