B02

Disruption of non-apoptotic Fas signalling disturbs B cell activation in autoimmune lymphoproliferative syndrome

Project Summary

Patients with Autoimmune lymphoproliferative syndrome (ALPS) suffer from autoantibody mediated autoimmunity and lymphoproliferation and carry mutations in the Fas gene. The Fas mutation leads to a defective germinal centre selection of B cells explaining the susceptibility to autoimmunity in these patients. In the second funding period, the impact of Fas mutations on non-apoptotic Fas signalling will be studied with a focus on B cell activation.