Barbara Vilen PhD

EMAIL: barb_vilen@med.unc.edu

PERSONAL WEBSITE: https://www.med.unc.edu/microimm/directory/barbara-vilen-phd/

PUBLICATIONS: Publications Link

My laboratory studies systemic lupus erythematosus (SLE), a heterogeneous autoimmune disease associated with tissue damage in skin, kidneys, lung, heart, brain, and vasculature. Murine and human studies in my laboratory identified a lysosome defect in multiple hematopoietic cell types in a murine model of lupus (MRL/lpr), and in active SLE patients. Lysosomes are intracellular organelles that function as the “gut” of cells, degrading “obsolete” intracellular material during autophagy, or degrading extracellular debris phagocytosed/ endocytosed through multiple surface receptors. In mice and humans, lysosomes containing undegraded IgG-immune complexes (IgG-ICs) are exocytosed to the periphery of the cell where they fuse with the plasma membrane, releasing lysosomal cargo into the milieu and leaving hydrophobic proteins or receptor-bound cargo associated with the plasma membrane. IgG-ICs that remain on the cell surface provide a source of high avidity nuclear self-antigens that activate autoreactive B cells and perpetuate autoantibody responses. Extracellular release of IgG-ICs elevates the levels of circulating immune complexes, which deposit in tissue and promote inflammation. Currently, we are exploring strategies to restore lysosome function as a potential therapeutic to maintain disease inactivity in SLE.