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Conventional therapies cause severe side effects and significant variation in efficacy, according to cohort studies. Thus, exploring new therapeutic approaches to prevent progression to liver failure is urgent. To study the physiology and pathology of WD, immortalized cell lines and rodent WD models have been used conventionally; however, a large gap remains among different species as well as in genetic backgrounds among individuals. We generated induced pluripotent stem cells (iPSCs) from four WD patients carrying compound heterozygous mutations in the ATP7B gene. ATP7B loss- and gain-of-functions were further manifested with ATP7B-deficient iPSCs and heterozygously corrected R778L WD patient-derived iPSCs using CRISPR-Cas9-based gene editing. Although the expression of ATP7B protein varied among WD-specific hepatocytes differentiated from these iPSCs, the expression and secretion of ceruloplasmin (Cp), a downstream copper carrier in plasma, were consistently decreased in WD patient-derived and ATP7B-deficient hepatocytes. A transcriptome analysis detected abnormalities in the retinoid signaling pathway and lipid metabolism in WD-specific hepatocytes. Drug screening using WD patient-derived hepatocytes identified retinoids as promising candidates for rescuing Cp secretion. All-trans retinoic acid also alleviates reactive oxygen species production induced by lipid accumulation in WD-specific hepatocytes treated with oleic acid. These patient-derived iPSC-based hepatic models function as effective platforms for the development of potential therapeutics for hepatic steatosis in WD and other fatty liver diseases.<\/jats:p>","DOI":"10.1093\/hmg\/ddac080","type":"journal-article","created":{"date-parts":[[2022,4,7]],"date-time":"2022-04-07T07:54:00Z","timestamp":1649318040000},"page":"3652-3671","source":"Crossref","is-referenced-by-count":21,"title":["Retinoids rescue ceruloplasmin secretion and alleviate oxidative stress in Wilson\u2019s disease-specific hepatocytes"],"prefix":"10.1093","volume":"31","author":[{"given":"Dan","family":"Song","sequence":"first","affiliation":[{"name":"iPS Cell Advanced Characterization and Development Team, RIKEN BioResource Research Center , Ibaraki 305-0074 , Japan"},{"name":"Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba , Ibaraki 305-8575 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gou","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Regenerative Medicine Project, Tokyo Metropolitan Institute of Medical Science , Tokyo 156-8506 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yun-Wen","family":"Zheng","sequence":"additional","affiliation":[{"name":"Guangdong Provincial Key Laboratory of Large Animal Models for Biomedicine, and School of Biotechnology and Heath Sciences, Wuyi University , Guangdong 529020 , China"},{"name":"Department of Medicinal and Life Sciences, Faculty of Pharmaceutical Sciences, Tokyo University of Science , Chiba 278-8510 , Japan"},{"name":"Department of Regenerative Medicine, Graduate School of Medicine, Yokohama City University , Kanagawa 234-0006 , Japan"},{"name":"Division of Regenerative Medicine, Center for Stem Cell Biology and Regenerative Medicine, Institute of Medical Science, The University of Tokyo , Tokyo 108-8639 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mami","family":"Matsuo-Takasaki","sequence":"additional","affiliation":[{"name":"iPS Cell Advanced Characterization and Development Team, RIKEN BioResource Research Center , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingyue","family":"Li","sequence":"additional","affiliation":[{"name":"iPS Cell Advanced Characterization and Development Team, RIKEN BioResource Research Center , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miho","family":"Takami","sequence":"additional","affiliation":[{"name":"iPS Cell Advanced Characterization and Development Team, RIKEN BioResource Research Center , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuri","family":"An","sequence":"additional","affiliation":[{"name":"iPS Cell Advanced Characterization and Development Team, RIKEN BioResource Research Center , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuko","family":"Hemmi","sequence":"additional","affiliation":[{"name":"iPS Cell Advanced Characterization and Development Team, RIKEN BioResource Research Center , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natsumi","family":"Miharada","sequence":"additional","affiliation":[{"name":"Cell Engineering Division, BioResource Research Center , RIKEN, Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsuyoshi","family":"Fujioka","sequence":"additional","affiliation":[{"name":"Cell Engineering Division, BioResource Research Center, RIKEN , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michiya","family":"Noguchi","sequence":"additional","affiliation":[{"name":"Cell Engineering Division, BioResource Research Center, RIKEN , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Nakajima","sequence":"additional","affiliation":[{"name":"National Hospital Organization, Niigata National Hospital , Niigata 945-8585 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megumu K","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Clinical Application, Center for iPS Cell Research and Application (CiRA), Kyoto University , Kyoto 606-8507 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukio","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Cell Engineering Division, BioResource Research Center, RIKEN , Ibaraki 305-0074 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuya","family":"Oda","sequence":"additional","affiliation":[{"name":"Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba , Ibaraki 305-8575 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