{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T13:03:45Z","timestamp":1791896625722,"version":"4.3.4"},"reference-count":0,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":7092,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1996,4]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Disruption of the murine <jats:italic toggle=\"yes\">mdr<\/jats:italic>2 (multidrug-resistance) gene, which encodes a phosphatidylcholine flippase, leads to a hepatic disorder because of loss of biliary phospholipid secretion. Among the hereditary human cholestasis, a subtype of progressive familial intrahepatic cholestasis with high gamma-glutamyltranspeptidase (GGT) serum activity shares histological, biochemical, and genetic features with mice lacking <jats:italic toggle=\"yes\">mdr<\/jats:italic>2 gene expression (<jats:italic toggle=\"yes\">mdr<\/jats:italic>2 -\/- mice). No <jats:italic toggle=\"yes\">mdr<\/jats:italic>3 (human <jats:italic toggle=\"yes\">mdr<\/jats:italic>2 homolog) messenger RNA (mRNA) was detected by Northern blotting in the liver of a patient suffering from this form of PFIC, and the biliary phospholipid level in a second patient was substantially decreased. Thus, the absence of the <jats:italic toggle=\"yes\">mdr<\/jats:italic>3 P-glycoprotein may be responsible for this type of PFIC, which, as in the murine model, may be due to a toxic effect of bile acids on the biliary epithelium in absence of biliary phospholipids.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.510230435","type":"journal-article","created":{"date-parts":[[2004,11,12]],"date-time":"2004-11-12T00:05:44Z","timestamp":1100217944000},"page":"904-908","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":256,"title":["Defect of multidrug-resistance 3 gene expression in a subtype of progressive familial intrahepatic cholestasis"],"prefix":"10.1097","volume":"23","author":[{"given":"J","family":"Deleuze","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"Jacquemin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C","family":"Dubuisson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D","family":"Cresteil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Dumont","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Erlinger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O","family":"Bernard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Hadchouel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","container-title":["Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhep.510230435","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01515467-199604000-00035","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,1]],"date-time":"2024-12-01T11:23:00Z","timestamp":1733052180000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01515467-199604000-00035"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1996,4]]},"references-count":0,"aliases":["10.1053\/jhep.1996.v23.pm0008666348"],"journal-issue":{"issue":"4","published-print":{"date-parts":[[1996]]}},"URL":"https:\/\/doi.org\/10.1002\/hep.510230435","relation":{},"ISSN":["0270-9139"],"issn-type":[{"value":"0270-9139","type":"print"}],"subject":[],"published":{"date-parts":[[1996,4]]}}}