Regulation of immune response and hepatic fibrosis by the cytotoxic effector molecules granzyme B and TRAIL in sclerosing cholangitis in mice

Background Primary sclerosing cholangitis (PSC) is a difficult to treat chronic inflammatory cholestatic liver disease. We observed an increased interferon (IFN)γ response in PSC patients and in a mouse model of sclerosing cholangitis. IFNγ induced expression of the cytotoxic effector molecules granzyme B (GzmB) and tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) in hepatic NK and CD8+T cells and mediated liver fibrosis in Mdr2-/- mice. Here, we investigated the significance of GzmB and TRAIL for PSC progression in Mdr2-/- mice.