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

Project Summary

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.