{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T19:49:26Z","timestamp":1783367366418,"version":"3.54.6"},"reference-count":35,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2018,5,28]],"date-time":"2018-05-28T00:00:00Z","timestamp":1527465600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2018,5,28]],"date-time":"2018-05-28T00:00:00Z","timestamp":1527465600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"name":"Vice Chancellor for Research, Tehran University of Medical Sciences","award":["1394-02-154-29492"],"award-info":[{"award-number":["1394-02-154-29492"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal Cellular Physiology"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:p>\n                    Patients with lipopolysaccharides responsive beige\u2010like anchor protein (LRBA) deficiency suffer from a variety of immunological abnormalities. In the current study, we investigated the role of T helper (Th) cell subsets and regulatory T (Treg) cells\u00a0and their related cytokines and transcription factors in the immune dysregulation of LRBA deficiency. The study population comprised of 13 LRBA\u2010deficient patients and 13 age\u2010 and\u00a0sex\u2010matched healthy controls (HCs). Th subsets and Treg were examined by flow cytometry. The expression of determinant cytokines (interferon\u2010\u03b3 [IFN\u2010\u03b3], interleukin [IL]\u201017, IL\u201022, and IL\u201010), and cell subset\u2013specific transcription factors were evaluated before and after proliferation and activation stimuli. The frequencies of Th1, Th1\u2010like Th17 and Th22 cells along with the expression of T\u2010box transcription factor (TBET) and runt\u2010related transcription factor 1 (RUNX1) were significantly increased in patients with LRBA. Moreover, IFN\u2010\u03b3 and IL\u201022 production in LRBA\u2010de\ufb01cient CD4\n                    <jats:sup>+<\/jats:sup>\n                    T cells were elevated after lymphocyte stimulation, particularly in patients with enteropathy. However, CD4\n                    <jats:sup>+<\/jats:sup>\n                    CD25\n                    <jats:sup>+<\/jats:sup>\n                    FoxP3\n                    <jats:sup>+<\/jats:sup>\n                    CD127\n                    <jats:sup>\u2212<\/jats:sup>\n                    cells were significantly decreased in LRBA\u2010deficient patients compared with those of HCs, particularly in patients with autoimmunity. There was a negative correlation between the frequencies of CD4\n                    <jats:sup>+<\/jats:sup>\n                    CD25\n                    <jats:sup>+<\/jats:sup>\n                    FoxP3\n                    <jats:sup>+<\/jats:sup>\n                    CD127\n                    <jats:sup>\u2212<\/jats:sup>\n                    cells and Th1\u2010like Th17 cells in LRBA\u2010deficient patients, and an overlapping phenotype of autoimmunity and enteropathy were observed in ~70% of patients. The frequency of Th17 cells was lower in patients with enteropathy, while Th1\u2010like Th17 cells were higher than in those without enteropathy. Our findings demonstrated an imbalance in Th subsets, mainly in Th1\u2010like Th17 and Treg cells and their corresponding cytokines in LRBA deficiency, which might be important in the immunopathogenesis of autoimmunity and enteropathy.\n                  <\/jats:p>","DOI":"10.1002\/jcp.26772","type":"journal-article","created":{"date-parts":[[2018,5,28]],"date-time":"2018-05-28T08:20:12Z","timestamp":1527495612000},"page":"8767-8777","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["The imbalance of circulating T helper subsets and regulatory T cells in patients with LRBA deficiency: Correlation with disease severity"],"prefix":"10.1002","volume":"233","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5658-2511","authenticated-orcid":false,"given":"Gholamreza","family":"Azizi","sequence":"first","affiliation":[{"name":"Non\u2010Communicable Diseases Research Center Alborz University of Medical Sciences  Karaj 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Education and Research Network (USERN)  Tehran Iran"},{"name":"Division of Clinical Immunology, Department of Laboratory Medicine Karolinska Institute at Karolinska University Hospital Huddinge  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reza","family":"Yazdani","sequence":"additional","affiliation":[{"name":"Research Center for Immunodeficiencies, Children\u2019s Medical Center Tehran University of Medical Sciences  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alireza","family":"Ghanavatinejad","sequence":"additional","affiliation":[{"name":"Department of Immunology, School of Public Health Tehran University of Medical Sciences  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Farshid","family":"Noorbakhsh","sequence":"additional","affiliation":[{"name":"Department of Immunology, School of Medicine Tehran University of Medical Sciences  Tehran 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