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However, the role of DN T\u2010cells in the pathogenesis of primary biliary cholangitis (PBC) is still unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We investigated the number and function of DN T\u2010cells in peripheral blood and liver biopsy specimens of PBC patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The number and frequency of DN T\u2010cells significantly decreased in peripheral blood and liver tissue of PBC patients. Furthermore, the frequency of DN T\u2010cells in PBC was negatively correlated with disease severity and positively correlated with ursodeoxycholic acid response. In vitro assays showed that perforin expression and the suppressive capability of DN T\u2010cells on the proliferation of CD4<jats:sup>+<\/jats:sup> and CD8<jats:sup>+<\/jats:sup> T\u2010cells were impaired in PBC. Finally, lithocholic acid, the most hydrophobic acid, could downregulate the proliferation and perforin expression of DN T\u2010cells.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Decreased quantity and function of DN T\u2010cells in PBC may result in the loss of immune regulations on effector CD4<jats:sup>+<\/jats:sup> and cytotoxic CD8<jats:sup>+<\/jats:sup> T\u2010cells, and thereby may break the immune tolerance and promote the pathogenesis of PBC.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.14136","type":"journal-article","created":{"date-parts":[[2019,5,14]],"date-time":"2019-05-14T17:03:42Z","timestamp":1557853422000},"page":"1755-1767","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Alteration of liver\u2010infiltrated and peripheral blood double\u2010negative T\u2010cells in primary biliary cholangitis"],"prefix":"10.1111","volume":"39","author":[{"given":"Shu 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