YA-HUI CHUANGLian Z.-X.Tsuneyama K.BOR-LUEN CHIANGAnsari A.A.Coppel R.L.Eric Gershwin M.2020-07-012020-07-0120060896-8411https://scholars.lib.ntu.edu.tw/handle/123456789/507649Although the pathogenesis of primary biliary cirrhosis (PBC) remains enigmatic, the immune system plays a key role in the initiation and subsequent development of pathology. Previous studies have indicated a critical role of the innate immune system. Importantly, natural killer (NK) cells are abundant in liver where they serve as sentinels of the immune system. In addition, NK cells have significant biologic activity based on their production of immunoregulatory cytokines. To address this issue, we have investigated several qualitative and quantitative activities of NK cells in patients with PBC as well as normal and liver diseased controls. We report herein a marked increase in the frequency and absolute number of blood and liver NK cells in PBC patients. Moreover, the cytotoxic activity and perforin expression by isolated NK cells were significantly increased in PBC patients associated with increased levels of plasma IL-8 and the expression of CD128a (IL-8 receptor) on NK cells. In contrast, the levels of IFN-γ, IL-6 and IL-8 synthesized by NK cells were significantly decreased in PBC patients as compared to controls. In conclusion, data from this study provide compelling evidence supporting a biologic role of NK cells in the immunopathogenesis of PBC. ? 2006 Elsevier Ltd. All rights reserved.[SDGs]SDG3gamma interferon; interleukin 6; interleukin 8; perforin; adult; aged; article; blood cell; blood level; cell count; cell isolation; cytokine production; cytotoxicity; data analysis; female; human; immunopathogenesis; liver cell; major clinical study; male; natural killer cell; primary biliary cirrhosis; priority journal; Adult; Aged; Cytokines; Female; Humans; Immunity, Natural; Interferon Type II; Interleukin-6; Interleukin-8; Killer Cells, Natural; Liver Cirrhosis, Biliary; Male; Membrane Glycoproteins; Middle Aged; Poly I-C; Pore Forming Cytotoxic Proteins; Receptors, Interleukin-8Ajournal article10.1016/j.jaut.2006.04.001167304272-s2.0-33746834534