{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T04:16:00Z","timestamp":1771647360101,"version":"3.50.1"},"reference-count":31,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,7,24]],"date-time":"2023-07-24T00:00:00Z","timestamp":1690156800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["HIT168002"],"award-info":[{"award-number":["HIT168002"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["PJT162098"],"award-info":[{"award-number":["PJT162098"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["PJT162298"],"award-info":[{"award-number":["PJT162298"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>ATP\u2010binding cassette transporters are important proteins in regulating bile constituent transport between hepatocytes and the bile canalicular system. Dysfunctional transporters lead to accumulation of toxic bile components within hepatocytes or the biliary system, known as cholestasis, resulting in liver damage. It has been previously reported that two particular ATP\u2010binding cassette transporters, ABCB4 and ABCB11, have altered expression in patients with primary sclerosing cholangitis (PSC). Interested in further analysis of expression patterns of ATP\u2010binding cassette transporters in PSC patients, we investigated liver samples from 201 patients, including 43 patients with PSC and 51 patients with primary biliary cholangitis patients (PBC). In addition to ABCB4 and ABCB11, we also included other ATP\u2010binding cassette transporters, to determine if upregulation of ABCB4 and ABCB11 is specifically found in the liver of patients with PSC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>Retrospectively, formalin\u2010fixed and paraffin\u2010embedded liver biopsies, resections, and explants were selected to investigate the expression of <jats:italic>ABCB1<\/jats:italic>, <jats:italic>ABCB4<\/jats:italic>, <jats:italic>ABCB11<\/jats:italic>, <jats:italic>ABCG5\/8<\/jats:italic>, and <jats:italic>FXR1<\/jats:italic> using nanoString nCounter and immunohistochemistry for validation of differently expressed transporters seen in PSC liver samples in comparison to non\u2010PSC liver specimens. Strikingly, ABCB4 was the only ATP\u2010binding cassette transporter showing increased gene and protein expression in hepatocytes of PSC livers when compared to non\u2010PSC liver specimens. Furthermore, ABCB4 protein expression also correlated with disease stage in PSC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our study concluded that altered ABCB4 expression is specifically seen in liver specimens of PSC patients. Therefore, quantitative ABCB4 analysis may be an additional useful tool for the histopathological diagnosis of PSC to distinguish this entity from other cholangiopathies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.15006","type":"journal-article","created":{"date-parts":[[2023,7,25]],"date-time":"2023-07-25T04:56:37Z","timestamp":1690260997000},"page":"559-568","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Analysis of various <scp>ATP<\/scp>\u2010binding cassette transporters revealed quantification of <scp>ABCB4<\/scp> as a potential diagnostic tool in primary sclerosing cholangitis (<scp>PSC<\/scp>)"],"prefix":"10.1111","volume":"83","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7860-8724","authenticated-orcid":false,"given":"Cornelia","family":"Thoeni","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catia T","family":"Perciani","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto ON Canada"},{"name":"Ajmera Transplant Centre Toronto General Hospital Research Institute  Toronto ON Canada"},{"name":"Department of Immunology University of Toronto, Medical Sciences Building  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diana","family":"Nakib","sequence":"additional","affiliation":[{"name":"Ajmera Transplant Centre Toronto General Hospital Research Institute  Toronto ON Canada"},{"name":"Department of Immunology University of Toronto, Medical Sciences Building  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Damra","family":"Camat","sequence":"additional","affiliation":[{"name":"Ajmera Transplant Centre Toronto General Hospital Research Institute  Toronto ON Canada"},{"name":"Department of Immunology University of Toronto, Medical Sciences Building  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian D","family":"McGilvray","sequence":"additional","affiliation":[{"name":"Ajmera Transplant Centre Toronto General Hospital Research Institute  Toronto ON Canada"},{"name":"Multi\u2010Organ Transplant Program Toronto General Hospital Research Institute  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonya A","family":"MacParland","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto ON Canada"},{"name":"Ajmera Transplant Centre Toronto General Hospital Research Institute  Toronto ON Canada"},{"name":"Department of Immunology University of Toronto, Medical Sciences Building  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandra","family":"Fischer","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto ON Canada"},{"name":"Department of Pathology, Laboratory Medicine Program University Health Network  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,7,24]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1128\/mr.57.4.995-1017.1993"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1124\/dmd.113.056358"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2009.11.012"},{"key":"e_1_2_11_5_1","first-page":"39","article-title":"A comprehensive review of progressive familial intrahepatic cholestasis (PFIC): genetic disorders of hepatocanalicular transporters","volume":"7","author":"Amer S","year":"2014","journal-title":"Gastroenterol. 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