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Failure of this system can lead to a buildup of conjugated bilirubin in the blood, resulting in jaundice.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>Hyperbilirubinemia is an important clinical sign that needs to be investigated under a stepwise evaluation. Inherited non\u2010hemolytic conjugated hyperbilirubinemic conditions include Dubin\u2010Johnson syndrome (caused by mutations affecting <jats:italic>ABCC2<\/jats:italic> gene) and Rotor syndrome (caused by the simultaneous presence of mutations in <jats:italic>SLCO1B1<\/jats:italic> and <jats:italic>SLCO1B3<\/jats:italic> genes). Although classically viewed as benign conditions requiring no treatment, they lately gained an increased interest since recent studies suggested that mutations in the responsible genes leading to hyperbilirubinemia, as well as minor genetic variants, may result in an increased susceptibility to drug toxicity.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>This article provides a comprehensive review on the pathophysiology of Dubin\u2010Johnson and Rotor syndromes, presenting the current knowledge concerning the molecular details and basis of these conditions.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ueg2.12279","type":"journal-article","created":{"date-parts":[[2022,7,21]],"date-time":"2022-07-21T06:40:47Z","timestamp":1658385647000},"page":"745-753","source":"Crossref","is-referenced-by-count":18,"title":["Benign inheritable disorders of bilirubin metabolism manifested by conjugated hyperbilirubinemia\u2013A narrative review"],"prefix":"10.1002","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6646-6960","authenticated-orcid":false,"given":"Mariana B.","family":"Morais","sequence":"first","affiliation":[{"name":"Centro Hospitalar Universit\u00e1rio Lisboa Norte Hospital de Santa Maria  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7769-7860","authenticated-orcid":false,"given":"Mariana Verdelho","family":"Machado","sequence":"additional","affiliation":[{"name":"Gastroenterology Department Faculdade de Medicina, Universidade de Lisboa  Lisbon Portugal"},{"name":"Gastroenterology Department Hospital de Vila Franca de Xira  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,7,20]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/fampra\/cmr118"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440\u20101746.17.s1.15.x"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0168\u20108278(99)80180\u20109"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.1997.272.6.g1285"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI117091"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.11.1.9034161"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.271.25.15091"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0163\u20107258(94)90034\u20105"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.12.6914"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.16.10046"},{"issue":"156","key":"e_1_2_13_12_1","first-page":"549","article-title":"Dubin\u2010Johnson syndrome in Israel. 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