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Innate lymphoid cells (ILCs) have been reported to play a role in liver homeostasis and autoimmunity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We evaluated the features of peripheral ILC1s and ILC3 in patients with PBC and hepatic ILC1 and ILC3 in two different PBC mouse models (dominant\u2010negative transforming growth factor\u2010beta receptor II [dnTGF\u03b2RII] and 2\u2010octynoic acid\u2010bovine serum albumin [2OA\u2010BSA]).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 115 patients and 18 healthy controls were enrolled in the study. Decreased circulating ILC1\/3s were observed in early\u2010stage PBC patients, and the numbers of ILC1\/3s were negatively correlated with specific parameters and the proportion of T\u2010helper (Th) 1 and Th17 cells. Reduced numbers of ILC1s were observed in PBC mouse models with different etiologies. ILC1\u2010deficient mice had more severe hepatic inflammation after inducing the 2OA\u2010BSA model. Continuous low\u2010dose injections of lipopolysaccharide (LPS) reduced ILC1 levels in mice, consistent with the lower level of ILC1s in PBC patients with high LPS (&gt;\u00a050\u00a0ng\/mL), and aggravated hepatic lymphocyte infiltration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients with PBC had decreased ILC1s, which were negatively correlated with CD4<jats:sup>+<\/jats:sup> T cells. Deficient ILC1 populations led to disease exacerbations in mice. Our results indicated that ILC1s may participate in the pathogenesis of PBC.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jgh.16547","type":"journal-article","created":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T08:48:35Z","timestamp":1712911715000},"page":"1431-1441","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Innate lymphoid cell subsets in the pathogenesis of primary biliary cholangitis"],"prefix":"10.1111","volume":"39","author":[{"given":"Yansheng","family":"Liu","sequence":"first","affiliation":[{"name":"State Key Laboratory of Holistic Integrative Management of Gastrointestinal Cancers and National Clinical Research Center for Digestive Diseases Xijing Hospital of Digestive Diseases, Fourth Military Medical University  Xi'an China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yinan","family":"Hu","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Holistic 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