Immune-mediated pathology as a consequence of impaired lymphocyte-extracellular matrix interactions

Project Summary

This project will be based on insights of the last funding period on altered B cell development during collagen VII deficiency. The functional implications of collagen VII deficiency for B cell development towards an autoimmune-associated phenotype will be further mechanistically interrogated. Given the demonstration of autoantibody-driven autoimmunity as a driver of advanced disease in dystrophic epidermolysis bullosa (DEB), B03 will also evaluate proteasome inhibition or neonatal Fc receptor antagonism as a therapeutic approach to control autoimmune- and inflammation-driven organ damage in DEB mice.