{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T07:35:32Z","timestamp":1791876932534,"version":"4.3.4"},"reference-count":152,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2022,8,18]],"date-time":"2022-08-18T00:00:00Z","timestamp":1660780800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Institute of Psychiatry and Neurology, Warsaw"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Wilson\u2019s disease (WD) is a hereditary disorder of copper metabolism, producing abnormally high levels of non-ceruloplasmin-bound copper, the determinant of the pathogenic process causing brain and hepatic damage and dysfunction. Although the disease is invariably fatal without medication, it is treatable and many of its adverse effects are reversible. Diagnosis is difficult due to the large range and severity of symptoms. A high index of suspicion is required as patients may have only a few of the many possible biomarkers. The genetic prevalence of ATP7B variants indicates higher rates in the population than are currently diagnosed. Treatments have evolved from chelators that reduce stored copper to zinc, which reduces the toxic levels of circulating non-ceruloplasmin-bound copper. Zinc induces intestinal metallothionein, which blocks copper absorption and increases excretion in the stools, resulting in an improvement in symptoms. Two meta-analyses and several large retrospective studies indicate that zinc is equally effective as chelators for the treatment of WD, with the advantages of a very low level of toxicity and only the minor side effect of gastric disturbance. Zinc is recommended as a first-line treatment for neurological presentations and is gaining acceptance for hepatic presentations. It is universally recommended for lifelong maintenance therapy and for presymptomatic WD.<\/jats:p>","DOI":"10.3390\/ijms23169316","type":"journal-article","created":{"date-parts":[[2022,8,18]],"date-time":"2022-08-18T21:39:21Z","timestamp":1660858761000},"page":"9316","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["The Role of Zinc in the Treatment of Wilson\u2019s Disease"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5537-6385","authenticated-orcid":false,"given":"Abolfazl","family":"Avan","sequence":"first","affiliation":[{"name":"Department of Public Health, School of Medicine, Mashhad University of Medical Sciences, Mashhad 93518-88415, Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1956-1866","authenticated-orcid":false,"given":"Anna","family":"Cz\u0142onkowska","sequence":"additional","affiliation":[{"name":"2nd Department of Neurology, Institute of Psychiatry and Neurology, 02-957 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2036-2124","authenticated-orcid":false,"given":"Susan","family":"Gaskin","sequence":"additional","affiliation":[{"name":"Department of Civil Engineering, McGill University, Montreal, QC H3A 0C3, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9181-4373","authenticated-orcid":false,"given":"Alberto","family":"Granzotto","sequence":"additional","affiliation":[{"name":"Center for Advanced Studies and Technology (CAST), University \u201cG. d\u2019Annunzio\u201d of Chieti-Pescara, 66100 Chieti, Italy"},{"name":"Department of Neuroscience, Imaging, and Clinical Sciences (DNISC), University \u201cG. d\u2019Annunzio\u201d of Chieti-Pescara, 66100 Chieti, Italy"},{"name":"Sue and Bill Gross Stem Cell Research Center, University of California-Irvine, Irvine, CA 92697, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0710-2574","authenticated-orcid":false,"given":"Stefano L.","family":"Sensi","sequence":"additional","affiliation":[{"name":"Center for Advanced Studies and Technology (CAST), University \u201cG. d\u2019Annunzio\u201d of Chieti-Pescara, 66100 Chieti, Italy"},{"name":"Department of Neuroscience, Imaging, and Clinical Sciences (DNISC), University \u201cG. d\u2019Annunzio\u201d of Chieti-Pescara, 66100 Chieti, Italy"},{"name":"Institute for Advanced Biomedical Technologies (ITAB), University \u201cG. d\u2019Annunzio\u201d of Chieti-Pescara, 66100 Chieti, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tjaard U.","family":"Hoogenraad","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Medical Centre Utrecht, 3584 CX Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.braindev.2005.08.008","article-title":"Paradigm shift in treatment of Wilson\u2019s disease: Zinc therapy now treatment of choice","volume":"28","author":"Hoogenraad","year":"2006","journal-title":"Brain Dev."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1136\/jnnp-2021-326123","article-title":"Wilson\u2019s disease: Update on pathogenesis, biomarkers and treatments","volume":"92","author":"Shribman","year":"2021","journal-title":"J. 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