{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T03:13:03Z","timestamp":1791947583328,"version":"4.3.4"},"reference-count":100,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,9,26]],"date-time":"2024-09-26T00:00:00Z","timestamp":1727308800000},"content-version":"am","delay-in-days":330,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["F32DK089689-01A1"],"award-info":[{"award-number":["F32DK089689-01A1"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["K01DK111716"],"award-info":[{"award-number":["K01DK111716"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["R01DK129579-01A1"],"award-info":[{"award-number":["R01DK129579-01A1"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007917","name":"Agricultural Research Service","doi-asserted-by":"publisher","award":["3092-51000-062-03S"],"award-info":[{"award-number":["3092-51000-062-03S"]}],"id":[{"id":"10.13039\/100007917","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Pharmacology &amp; Therapeutics"],"published-print":{"date-parts":[[2023,11]]},"DOI":"10.1016\/j.pharmthera.2023.108529","type":"journal-article","created":{"date-parts":[[2023,9,22]],"date-time":"2023-09-22T00:01:31Z","timestamp":1695340891000},"page":"108529","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":16,"special_numbering":"C","title":["Therapeutic implications of impaired nuclear receptor function and dysregulated metabolism in Wilson's disease"],"prefix":"10.1016","volume":"251","author":[{"given":"Clavia Ruth","family":"Wooton-Kee","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"1\u20132","key":"10.1016\/j.pharmthera.2023.108529_bb0005","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/j.molbrainres.2005.02.025","article-title":"A new strain of rat for functional analysis of PINA","volume":"137","author":"Ahmed","year":"2005","journal-title":"Brain Research. Molecular Brain Research"},{"issue":"31","key":"10.1016\/j.pharmthera.2023.108529_bb0010","doi-asserted-by":"crossref","first-page":"28857","DOI":"10.1074\/jbc.M011610200","article-title":"Human bile salt export pump promoter is transactivated by the farnesoid X receptor\/bile acid receptor","volume":"276","author":"Ananthanarayanan","year":"2001","journal-title":"The Journal of Biological Chemistry"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0015","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/j.hepres.2004.10.009","article-title":"Liver cirrhosis and metabolism (sugar, protein, fat and trace elements)","volume":"30S","author":"Arakawa","year":"2004","journal-title":"Hepatology Research"},{"issue":"19","key":"10.1016\/j.pharmthera.2023.108529_bb0020","doi-asserted-by":"crossref","first-page":"6803","DOI":"10.1073\/pnas.0800019105","article-title":"Mitochondrial copper(I) transfer from Cox17 to Sco1 is coupled to electron transfer","volume":"105","author":"Banci","year":"2008","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0025","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/B978-0-444-63625-6.00009-4","article-title":"Hepatic features of Wilson disease","volume":"142","author":"Boga","year":"2017","journal-title":"Handbook of Clinical Neurology"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0030","doi-asserted-by":"crossref","first-page":"1018","DOI":"10.1523\/JNEUROSCI.19-03-01018.1999","article-title":"A novel pineal night-specific ATPase encoded by the Wilson disease gene","volume":"19","author":"Borjigin","year":"1999","journal-title":"The Journal of Neuroscience"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0035","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1016\/S0022-2143(99)90213-5","article-title":"Treatment of Wilson\u2019s disease with zinc","volume":"134","author":"Brewer","year":"1999","journal-title":"The Journal of Laboratory and Clinical Medicine"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0040","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1111\/j.1572-0241.1999.00301.x","article-title":"Zinc therapy induction of intestinal metallothionein in Wilson\u2019s disease","volume":"94","author":"Brewer","year":"1999","journal-title":"The American Journal of Gastroenterology"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0045","doi-asserted-by":"crossref","first-page":"144","DOI":"10.7326\/0003-4819-101-1-144_3","article-title":"Zinc treatment of Wilson\u2019s disease","volume":"101","author":"Brewer","year":"1984","journal-title":"Annals of Internal Medicine"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0050","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1001\/archneur.60.3.379","article-title":"Treatment of Wilson disease with ammonium tetrathiomolybdate: III. Initial therapy in a total of 55 neurologically affected patients and follow-up with zinc therapy","volume":"60","author":"Brewer","year":"2003","journal-title":"Archives of Neurology"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0055","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1053\/j.gastro.2005.10.054","article-title":"ATP7B mediates vesicular sequestration of copper: Insight into biliary copper excretion","volume":"130","author":"Cater","year":"2006","journal-title":"Gastroenterology"},{"issue":"9","key":"10.1016\/j.pharmthera.2023.108529_bb0060","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.1016\/S0022-2275(20)30272-8","article-title":"Nuclear receptor-mediated repression of human cholesterol 7alpha-hydroxylase gene transcription by bile acids","volume":"42","author":"Chen","year":"2001","journal-title":"Journal of Lipid Research"},{"issue":"10","key":"10.1016\/j.pharmthera.2023.108529_bb0065","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1194\/jlr.R900010-JLR200","article-title":"Bile acids: Regulation of synthesis","volume":"50","author":"Chiang","year":"2009","journal-title":"Journal of Lipid Research"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0070","doi-asserted-by":"crossref","first-page":"111618","DOI":"10.1016\/j.mce.2022.111618","article-title":"Discovery of farnesoid X receptor and its role in bile acid metabolism","volume":"548","author":"Chiang","year":"2022","journal-title":"Molecular and Cellular Endocrinology"},{"issue":"Pt 8","key":"10.1016\/j.pharmthera.2023.108529_bb0075","doi-asserted-by":"crossref","first-page":"1997","DOI":"10.1093\/brain\/awp193","article-title":"Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, by S. A. Kinnier Wilson (From the National Hospital, and the Laboratory of the National Hospital, Queen Square, London). Brain 1912: 34; 295-509","volume":"132","author":"Compston","year":"2009","journal-title":"Brain"},{"issue":"10","key":"10.1016\/j.pharmthera.2023.108529_bb0080","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1007\/s00335-001-3021-y","article-title":"The Jackson toxic milk mouse as a model for copper loading","volume":"12","author":"Coronado","year":"2001","journal-title":"Mammalian Genome"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0085","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1111\/j.1399-0004.2005.00524.x","article-title":"COMMD1 (MURR1) as a candidate in patients with copper storage disease of undefined etiology","volume":"68","author":"Coronado","year":"2005","journal-title":"Clinical Genetics"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0090","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1002\/jmr.1148","article-title":"Structural and thermodynamic consequences of the replacement of zinc with environmental metals on estrogen receptor alpha-DNA interactions","volume":"24","author":"Deegan","year":"2011","journal-title":"Journal of Molecular Recognition"},{"issue":"42","key":"10.1016\/j.pharmthera.2023.108529_bb0095","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.adc9022","article-title":"Oxysterol misbalance critically contributes to Wilson disease pathogenesis","volume":"8","author":"Dev","year":"2022","journal-title":"Science Advances"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0100","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/j.jss.2009.10.006","article-title":"Nitric oxide and redox regulation in the liver: Part II. Redox biology in pathologic hepatocytes and implications for intervention","volume":"167","author":"Diesen","year":"2011","journal-title":"The Journal of Surgical Research"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0105","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1016\/j.jcmgh.2018.12.005","article-title":"A high-calorie diet aggravates mitochondrial dysfunction and triggers severe liver damage in Wilson disease rats","volume":"7","author":"Einer","year":"2019","journal-title":"Cellular and Molecular Gastroenterology and Hepatology"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0110","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1053\/j.gastro.2023.03.216","article-title":"ARBM101 (Methanobactin SB2) drains excess liver copper via biliary excretion in Wilson\u2019s disease rats","volume":"165","author":"Einer","year":"2023","journal-title":"Gastroenterology"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0115","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1080\/01652176.2011.563146","article-title":"Copper-induced hepatitis: The COMMD1 deficient dog as a translational animal model for human chronic hepatitis","volume":"31","author":"Favier","year":"2011","journal-title":"The Veterinary Quarterly"},{"issue":"8","key":"10.1016\/j.pharmthera.2023.108529_bb0120","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0042158","article-title":"COMMD1-deficient dogs accumulate copper in hepatocytes and provide a good model for chronic hepatitis and fibrosis","volume":"7","author":"Favier","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0125","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1111\/nyas.12442","article-title":"New canine models of copper toxicosis: Diagnosis, treatment, and genetics","volume":"1314","author":"Fieten","year":"2014","journal-title":"Annals of the New York Academy of Sciences"},{"issue":"12","key":"10.1016\/j.pharmthera.2023.108529_bb0130","doi-asserted-by":"crossref","first-page":"3486","DOI":"10.2337\/diabetes.51.12.3486","article-title":"Mechanisms of the triglyceride- and cholesterol-lowering effect of fenofibrate in hyperlipidemic type 2 diabetic patients","volume":"51","author":"Forcheron","year":"2002","journal-title":"Diabetes"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0135","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.ajpath.2021.09.010","article-title":"Hepatic steatosis in the mouse model of Wilson disease coincides with a muted inflammatory response","volume":"192","author":"Gottlieb","year":"2022","journal-title":"The American Journal of Pathology"},{"issue":"9228","key":"10.1016\/j.pharmthera.2023.108529_bb0140","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1016\/S0140-6736(00)02556-3","article-title":"Oxidative-phosphorylation defects in liver of patients with Wilson\u2019s disease","volume":"356","author":"Gu","year":"2000","journal-title":"Lancet"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0145","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1186\/1744-9081-6-33","article-title":"A novel COMMD1 mutation Thr174Met associated with elevated urinary copper and signs of enhanced apoptotic cell death in a Wilson disease patient","volume":"6","author":"Gupta","year":"2010","journal-title":"Behavioral and Brain Functions"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0150","doi-asserted-by":"crossref","first-page":"1903","DOI":"10.1007\/s11033-021-06192-5","article-title":"Toxic milk mice models of Wilson\u2019s disease","volume":"48","author":"Hadrian","year":"2021","journal-title":"Molecular Biology Reports"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0155","doi-asserted-by":"crossref","first-page":"1828","DOI":"10.1002\/hep.28406","article-title":"Activation of LXR\/RXR pathway ameliorates liver disease in atp7b (Wilson disease) mice","volume":"63","author":"Hamilton","year":"2015","journal-title":"Hepatology"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0160","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1016\/0014-4800(85)90041-3","article-title":"Copper toxicosis and tolerance in the rat. III. Intracellular localization of copper in the liver and kidney","volume":"43","author":"Haywood","year":"1985","journal-title":"Experimental and Molecular Pathology"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0165","first-page":"856","article-title":"Copper-associated chronic hepatitis in Labrador retrievers","volume":"20","author":"Hoffmann","year":"2006","journal-title":"Journal of Veterinary Internal Medicine"},{"issue":"8102","key":"10.1016\/j.pharmthera.2023.108529_bb0170","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1016\/S0140-6736(78)92141-4","article-title":"Oral zinc in Wilson's disease","volume":"2","author":"Hoogenraad","year":"1978","journal-title":"Lancet"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0175","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/S0021-9975(08)80268-X","article-title":"The pathology and trace element status of the toxic milk mutant mouse","volume":"110","author":"Howell","year":"1994","journal-title":"Journal of Comparative Pathology"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0180","doi-asserted-by":"crossref","first-page":"423","DOI":"10.2353\/ajpath.2006.050312","article-title":"Consequences of copper accumulation in the livers of the Atp7b\u2212\/\u2212 (Wilson disease gene) knockout mice","volume":"168","author":"Huster","year":"2006","journal-title":"The American Journal of Pathology"},{"issue":"11","key":"10.1016\/j.pharmthera.2023.108529_bb0185","doi-asserted-by":"crossref","first-page":"8343","DOI":"10.1074\/jbc.M607496200","article-title":"High copper selectively alters lipid metabolism and cell cycle machinery in the mouse model of Wilson disease","volume":"282","author":"Huster","year":"2007","journal-title":"The Journal of Biological Chemistry"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0190","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1001\/archinte.1972.00320040063007","article-title":"Glucose intolerance in Wilson\u2019s disease. Normalization after treatment with penicillamine","volume":"129","author":"Johansen","year":"1972","journal-title":"Archives of Internal Medicine"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0195","first-page":"614","article-title":"Prevention of spontaneous hepatocellular carcinoma in long-Evans cinnamon rats with hereditary hepatitis by the administration of D-penicillamine","volume":"18","author":"Jong-Hon","year":"1993","journal-title":"Hepatology"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0200","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1002\/hep.23090","article-title":"Zinc supplementation reverses alcohol-induced steatosis in mice through reactivating hepatocyte nuclear factor-4alpha and peroxisome proliferator-activated receptor-alpha","volume":"50","author":"Kang","year":"2009","journal-title":"Hepatology"},{"issue":"10","key":"10.1016\/j.pharmthera.2023.108529_bb0205","doi-asserted-by":"crossref","first-page":"1720","DOI":"10.1210\/mend.15.10.0712","article-title":"Farnesoid X-activated receptor induces apolipoprotein C-II transcription: A molecular mechanism linking plasma triglyceride levels to bile acids","volume":"15","author":"Kast","year":"2001","journal-title":"Molecular Endocrinology"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0210","doi-asserted-by":"crossref","first-page":"2908","DOI":"10.1074\/jbc.M109326200","article-title":"Regulation of multidrug resistance-associated protein 2 (ABCC2) by the nuclear receptors pregnane X receptor, farnesoid X-activated receptor, and constitutive androstane receptor","volume":"277","author":"Kast","year":"2002","journal-title":"The Journal of Biological Chemistry"},{"issue":"12","key":"10.1016\/j.pharmthera.2023.108529_bb0215","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1002\/humu.20586","article-title":"Sequence variation database for the Wilson disease copper transporter, ATP7B","volume":"28","author":"Kenney","year":"2007","journal-title":"Human Mutation"},{"issue":"12","key":"10.1016\/j.pharmthera.2023.108529_bb0220","doi-asserted-by":"crossref","first-page":"2664","DOI":"10.1194\/jlr.M700330-JLR200","article-title":"Differential regulation of bile acid homeostasis by the farnesoid X receptor in liver and intestine","volume":"48","author":"Kim","year":"2007","journal-title":"Journal of Lipid Research"},{"issue":"49","key":"10.1016\/j.pharmthera.2023.108529_bb0225","doi-asserted-by":"crossref","first-page":"41334","DOI":"10.1074\/jbc.M112.421834","article-title":"Nuclear receptors HNF4alpha and LRH-1 cooperate in regulating Cyp7a1 in vivo","volume":"287","author":"Kir","year":"2012","journal-title":"The Journal of Biological Chemistry"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0230","doi-asserted-by":"crossref","first-page":"1034","DOI":"10.1002\/hep.25740","article-title":"Mechanism of tissue-specific farnesoid X receptor in suppressing the expression of genes in bile-acid synthesis in mice","volume":"56","author":"Kong","year":"2012","journal-title":"Hepatology"},{"issue":"Suppl. 2","key":"10.1016\/j.pharmthera.2023.108529_bb0235","doi-asserted-by":"crossref","first-page":"S71","DOI":"10.21037\/atm.2019.02.06","article-title":"Preclinical models of Wilson\u2019s disease, why dogs are catchy alternatives","volume":"7","author":"Kruitwagen","year":"2019","journal-title":"Annals of Translational Medicine: Home"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0240","first-page":"249","article-title":"Endocrine symptoms as the initial manifestation of Wilson\u2019s disease","volume":"85","author":"Krysiak","year":"2012","journal-title":"The Yale Journal of Biology and Medicine"},{"issue":"5","key":"10.1016\/j.pharmthera.2023.108529_bb0245","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1039\/C3MT00366C","article-title":"Understanding copper sensitivity in zebrafish (Danio rerio) through the intracellular localization of copper transporters in a hepatocyte cell-line ZFL and the tissue expression profiles of copper transporters","volume":"6","author":"Leung","year":"2014","journal-title":"Metallomics"},{"issue":"16","key":"10.1016\/j.pharmthera.2023.108529_bb0250","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.1016\/j.lfs.2007.01.017","article-title":"Abnormal hepatobiliary and circulating lipid metabolism in the long-Evans cinnamon rat model of Wilson\u2019s disease","volume":"80","author":"Levy","year":"2007","journal-title":"Life Sciences"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0255","doi-asserted-by":"crossref","first-page":"1571","DOI":"10.1007\/s10695-022-01127-8","article-title":"Lipid-related metabolism during zebrafish embryogenesis under unbalanced copper homeostasis","volume":"48","author":"Li","year":"2022","journal-title":"Fish Physiology and Biochemistry"},{"issue":"5","key":"10.1016\/j.pharmthera.2023.108529_bb0260","doi-asserted-by":"crossref","first-page":"1858","DOI":"10.1172\/JCI115208","article-title":"Spontaneous hepatic copper accumulation in long-Evans cinnamon rats with hereditary hepatitis. A model of Wilson\u2019s disease","volume":"87","author":"Li","year":"1991","journal-title":"The Journal of Clinical Investigation"},{"issue":"7","key":"10.1016\/j.pharmthera.2023.108529_bb0265","doi-asserted-by":"crossref","first-page":"2721","DOI":"10.1172\/JCI85226","article-title":"Methanobactin reverses acute liver failure in a rat model of Wilson disease","volume":"126","author":"Lichtmannegger","year":"2016","journal-title":"The Journal of Clinical Investigation"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0270","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1007\/s00535-006-1807-0","article-title":"The canine copper toxicosis gene MURR1 is not implicated in the pathogenesis of Wilson disease","volume":"41","author":"Lovicu","year":"2006","journal-title":"Journal of Gastroenterology"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0275","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1152\/physrev.00004.2006","article-title":"Function and regulation of human copper-transporting ATPases","volume":"87","author":"Lutsenko","year":"2007","journal-title":"Physiological Reviews"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0280","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1172\/JCI25604","article-title":"Farnesoid X receptor is essential for normal glucose homeostasis","volume":"116","author":"Ma","year":"2006","journal-title":"The Journal of Clinical Investigation"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0285","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1016\/0092-8674(95)90199-X","article-title":"The nuclear receptor superfamily: The second decade","volume":"83","author":"Mangelsdorf","year":"1995","journal-title":"Cell"},{"issue":"10","key":"10.1016\/j.pharmthera.2023.108529_bb0290","doi-asserted-by":"crossref","first-page":"165842","DOI":"10.1016\/j.bbadis.2020.165842","article-title":"Activation of HIF-1 signaling ameliorates liver steatosis in zebrafish atp7b deficiency (Wilson\u2019s disease) models","volume":"2020","author":"Mi","year":"1866","journal-title":"Biochimica et Biophysica Acta - Molecular Basis of Disease"},{"issue":"5","key":"10.1016\/j.pharmthera.2023.108529_bb0295","first-page":"657","article-title":"Stimulation of liver fibrosis by N2 neutrophils in Wilson\u2019s disease. Cellular and Molecular Gastroenterology and","volume":"16","author":"Mi","year":"2023","journal-title":"Hepatology"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0300","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1186\/s13072-019-0255-z","article-title":"Epigenomic signatures in liver and blood of Wilson disease patients include hypermethylation of liver-specific enhancers","volume":"12","author":"Mordaunt","year":"2019","journal-title":"Epigenetics & Chromatin"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0305","doi-asserted-by":"crossref","first-page":"G39","DOI":"10.1152\/ajpgi.00312.2016","article-title":"Targeted inactivation of copper transporter Atp7b in hepatocytes causes liver steatosis and obesity in mice","volume":"313","author":"Muchenditsi","year":"2017","journal-title":"American Journal of Physiology. Gastrointestinal and Liver Physiology"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0310","doi-asserted-by":"crossref","first-page":"5659","DOI":"10.1038\/s41598-021-84894-3","article-title":"Systemic deletion of Atp7b modifies the hepatocytes\u2019 response to copper overload in the mouse models of Wilson disease","volume":"11","author":"Muchenditsi","year":"2021","journal-title":"Scientific Reports"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0315","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1203\/01.pdr.0000238341.12229.d3","article-title":"Relationship between oxidative stress and antioxidant systems in the liver of patients with Wilson disease: Hepatic manifestation in Wilson disease as a consequence of augmented oxidative stress","volume":"60","author":"Nagasaka","year":"2006","journal-title":"Pediatric Research"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0320","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1016\/j.ymgme.2012.08.004","article-title":"Fatty liver and anti-oxidant enzyme activities along with peroxisome proliferator-activated receptors gamma and alpha expressions in the liver of Wilson\u2019s disease","volume":"107","author":"Nagasaka","year":"2012","journal-title":"Molecular Genetics and Metabolism"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0325","first-page":"443","article-title":"Reference limits for copper and iron in liver biopsies","volume":"33","author":"Nuttall","year":"2003","journal-title":"Annals of Clinical and Laboratory Science"},{"issue":"5","key":"10.1016\/j.pharmthera.2023.108529_bb0330","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1002\/hep.1840150532","article-title":"Hepatocellular carcinoma: Recent progress","volume":"15","author":"Okuda","year":"1992","journal-title":"Hepatology"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0335","doi-asserted-by":"crossref","first-page":"111678","DOI":"10.1016\/j.mce.2022.111678","article-title":"Recent advances on FXR-targeting therapeutics","volume":"552","author":"Panzitt","year":"2022","journal-title":"Molecular and Cellular Endocrinology"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0340","doi-asserted-by":"crossref","DOI":"10.1053\/j.gastro.2017.09.019","article-title":"The function of ATPase copper transporter ATP7B in intestine","volume":"154","author":"Pierson","year":"2018","journal-title":"Gastroenterology"},{"issue":"12","key":"10.1016\/j.pharmthera.2023.108529_bb0345","doi-asserted-by":"crossref","DOI":"10.3390\/cancers11121852","article-title":"Structure and function of the nuclear receptor superfamily and current targeted therapies of prostate cancer","volume":"11","author":"Porter","year":"2019","journal-title":"Cancers (Basel)"},{"issue":"9","key":"10.1016\/j.pharmthera.2023.108529_bb0350","doi-asserted-by":"crossref","first-page":"5842","DOI":"10.1016\/S0021-9258(18)42630-0","article-title":"Effect of replacement of \u201czinc finger\u201d zinc on estrogen receptor DNA interactions","volume":"267","author":"Predki","year":"1992","journal-title":"The Journal of Biological Chemistry"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0355","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1006\/geno.1995.9968","article-title":"The toxic milk mutation, tx, which results in a condition resembling Wilson disease in humans, is linked to mouse chromosome 8","volume":"29","author":"Rauch","year":"1995","journal-title":"Genomics"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0360","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/j.ymgme.2007.08.127","article-title":"Mitochondrial structure and function in the untreated Jackson toxic milk (tx-j) mouse, a model for Wilson disease","volume":"93","author":"Roberts","year":"2008","journal-title":"Molecular Genetics and Metabolism"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0365","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1146\/annurev.biochem.72.121801.161712","article-title":"The enzymes, regulation, and genetics of bile acid synthesis","volume":"72","author":"Russell","year":"2003","journal-title":"Annual Review of Biochemistry"},{"issue":"2","key":"10.1016\/j.pharmthera.2023.108529_bb0370","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1002\/hep.30911","article-title":"The prevalence of Wilson\u2019s disease: An update","volume":"71","author":"Sandahl","year":"2020","journal-title":"Hepatology"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0375","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1007\/s11306-019-1505-6","article-title":"Metabolomics profiles of patients with Wilson disease reveal a distinct metabolic signature","volume":"15","author":"Sarode","year":"2019","journal-title":"Metabolomics"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0380","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1016\/j.jcmgh.2021.05.020","article-title":"Wilson disease: Intersecting DNA methylation and histone acetylation regulation of gene expression in a mouse model of hepatic copper accumulation","volume":"12","author":"Sarode","year":"2021","journal-title":"Cellular and Molecular Gastroenterology and Hepatology"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0385","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1002\/(SICI)1099-0461(2000)14:4<210::AID-JBT5>3.0.CO;2-G","article-title":"Biliary copper excretion capacity in intact animals: Correlation between ATP7B function, hepatic mass, and biliary copper excretion","volume":"14","author":"Schilsky","year":"2000","journal-title":"Journal of Biochemical and Molecular Toxicology"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0390","doi-asserted-by":"crossref","first-page":"1428","DOI":"10.1002\/hep.32805","article-title":"A multidisciplinary approach to the diagnosis and management of Wilson disease: 2022 practice guidance on Wilson disease from the American Association for the Study of Liver Diseases","volume":"77","author":"Schilsky","year":"2023","journal-title":"Hepatology"},{"issue":"42","key":"10.1016\/j.pharmthera.2023.108529_bb0395","doi-asserted-by":"crossref","first-page":"39411","DOI":"10.1074\/jbc.M106340200","article-title":"Disrupted bile acid homeostasis reveals an unexpected interaction among nuclear hormone receptors, transporters, and cytochrome P450","volume":"276","author":"Schuetz","year":"2001","journal-title":"The Journal of Biological Chemistry"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0400","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1210\/er.2018-00134","article-title":"Fibroblast growth factor 15\/19: From basic functions to therapeutic perspectives","volume":"39","author":"Somm","year":"2018","journal-title":"Endocrine Reviews"},{"issue":"3","key":"10.1016\/j.pharmthera.2023.108529_bb0405","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1016\/S0016-5085(19)34045-4","article-title":"Mitochondrial and fatty changes in hepatocytes of patients with Wilson\u2019s disease","volume":"55","author":"Sternlieb","year":"1968","journal-title":"Gastroenterology"},{"issue":"9","key":"10.1016\/j.pharmthera.2023.108529_bb0410","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1007\/s00109-004-0557-9","article-title":"Analysis of the human homologue of the canine copper toxicosis gene MURR1 in Wilson disease patients","volume":"82","author":"Stuehler","year":"2004","journal-title":"Journal of Molecular Medicine (Berlin, Germany)"},{"issue":"6586","key":"10.1016\/j.pharmthera.2023.108529_bb0415","doi-asserted-by":"crossref","first-page":"1254","DOI":"10.1126\/science.abf0529","article-title":"Copper induces cell death by targeting lipoylated TCA cycle proteins","volume":"375","author":"Tsvetkov","year":"2022","journal-title":"Science"},{"issue":"8","key":"10.1016\/j.pharmthera.2023.108529_bb0420","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1038\/s41574-018-0037-x","article-title":"Liver X receptors in lipid signalling and membrane homeostasis","volume":"14","author":"Wang","year":"2018","journal-title":"Nature Reviews. Endocrinology"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0425","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1010558","article-title":"Atp7b-dependent choroid plexus dysfunction causes transient copper deficit and metabolic changes in the developing mouse brain","volume":"19","author":"Washington-Hughes","year":"2023","journal-title":"PLoS Genetics"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0430","series-title":"GeneReviews((R))","article-title":"Wilson disease","author":"Weiss","year":"1993"},{"issue":"14","key":"10.1016\/j.pharmthera.2023.108529_bb0435","doi-asserted-by":"crossref","first-page":"2239","DOI":"10.3748\/wjg.v12.i14.2239","article-title":"Copper toxicosis gene MURR1 is not changed in Wilson disease patients with normal blood ceruloplasmin levels","volume":"12","author":"Weiss","year":"2006","journal-title":"World Journal of Gastroenterology"},{"issue":"7","key":"10.1016\/j.pharmthera.2023.108529_bb0440","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1039\/c2mt20017a","article-title":"A systems approach implicates nuclear receptor targeting in the Atp7b(\u2212\/\u2212) mouse model of Wilson\u2019s disease","volume":"4","author":"Wilmarth","year":"2012","journal-title":"Metallomics"},{"issue":"9","key":"10.1016\/j.pharmthera.2023.108529_bb0445","doi-asserted-by":"crossref","first-page":"3449","DOI":"10.1172\/JCI78991","article-title":"Elevated copper impairs hepatic nuclear receptor function in Wilson\u2019s disease","volume":"125","author":"Wooton-Kee","year":"2015","journal-title":"The Journal of Clinical Investigation"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0450","doi-asserted-by":"crossref","first-page":"2076","DOI":"10.1073\/pnas.1914267117","article-title":"Metabolic dysregulation in the Atp7b (\u2212\/\u2212) Wilson\u2019s disease mouse model","volume":"117","author":"Wooton-Kee","year":"2020","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0455","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1038\/ng0894-541","article-title":"The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene","volume":"7","author":"Wu","year":"1994","journal-title":"Nature Genetics"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0460","doi-asserted-by":"crossref","DOI":"10.3390\/vetsci6040078","article-title":"Reduced FXR target gene expression in copper-laden livers of COMMD1-deficient dogs","volume":"6","author":"Wu","year":"2019","journal-title":"Veterinary Sciences"},{"issue":"5","key":"10.1016\/j.pharmthera.2023.108529_bb0465","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1007\/s00109-005-0036-y","article-title":"Mutation analysis of 218 Chinese patients with Wilson disease revealed no correlation between the canine copper toxicosis gene MURR1 and Wilson disease","volume":"84","author":"Wu","year":"2006","journal-title":"Journal of Molecular Medicine (Berlin, Germany)"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0470","article-title":"1H NMR-based metabolomics investigation of copper-laden rat: A model of Wilson\u2019s disease","volume":"10","author":"Xu","year":"2015","journal-title":"PLoS One"},{"issue":"6","key":"10.1016\/j.pharmthera.2023.108529_bb0475","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1093\/oxfordjournals.jhered.a110416","article-title":"New mutation causing hereditary hepatitis in the laboratory rat","volume":"78","author":"Yoshida","year":"1987","journal-title":"The Journal of Heredity"},{"issue":"45","key":"10.1016\/j.pharmthera.2023.108529_bb0480","doi-asserted-by":"crossref","first-page":"41690","DOI":"10.1074\/jbc.M105117200","article-title":"Transcriptional regulation of the human sterol 12alpha-hydroxylase gene (CYP8B1): Roles of heaptocyte nuclear factor 4alpha in mediating bile acid repression","volume":"276","author":"Zhang","year":"2001","journal-title":"The Journal of Biological Chemistry"},{"issue":"1","key":"10.1016\/j.pharmthera.2023.108529_bb0485","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1038\/s41467-019-14138-6","article-title":"SUMOylation inhibitors synergize with FXR agonists in combating liver fibrosis","volume":"11","author":"Zhou","year":"2020","journal-title":"Nature Communications"},{"key":"10.1016\/j.pharmthera.2023.108529_bb0490","doi-asserted-by":"crossref","first-page":"113577","DOI":"10.1016\/j.biopha.2022.113577","article-title":"A new perspective on NAFLD: Focusing on the crosstalk between peroxisome proliferator-activated receptor alpha (PPARalpha) and farnesoid X receptor (FXR)","volume":"154","author":"Zhou","year":"2022","journal-title":"Biomedicine & Pharmacotherapy"},{"issue":"13","key":"10.1016\/j.pharmthera.2023.108529_bb0495","doi-asserted-by":"crossref","first-page":"5051","DOI":"10.1021\/ac800173r","article-title":"Electrophoretic analysis of the mitochondrial outer membrane rupture induced by permeability transition","volume":"80","author":"Zischka","year":"2008","journal-title":"Analytical Chemistry"},{"issue":"4","key":"10.1016\/j.pharmthera.2023.108529_bb0500","doi-asserted-by":"crossref","first-page":"1508","DOI":"10.1172\/JCI45401","article-title":"Liver mitochondrial membrane crosslinking and destruction in a rat model of Wilson disease","volume":"121","author":"Zischka","year":"2011","journal-title":"The Journal of Clinical Investigation"}],"container-title":["Pharmacology &amp; Therapeutics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0163725823001936?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0163725823001936?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,27]],"date-time":"2025-09-27T22:36:03Z","timestamp":1759012563000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0163725823001936"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11]]},"references-count":100,"alternative-id":["S0163725823001936"],"URL":"https:\/\/doi.org\/10.1016\/j.pharmthera.2023.108529","relation":{},"ISSN":["0163-7258"],"issn-type":[{"value":"0163-7258","type":"print"}],"subject":[],"published":{"date-parts":[[2023,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Therapeutic implications of impaired nuclear receptor function and dysregulated metabolism in Wilson's disease","name":"articletitle","label":"Article Title"},{"value":"Pharmacology & Therapeutics","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.pharmthera.2023.108529","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}],"article-number":"108529"}}