{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,6,5]],"date-time":"2024-06-05T15:03:39Z","timestamp":1717599819762},"reference-count":16,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2004,12,14]],"date-time":"2004-12-14T00:00:00Z","timestamp":1102982400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,12,14]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Wilson disease (WD) is an autosomal recessive disorder of copper transport, resulting in copper accumulation and toxicity to the liver and brain. There is no evidence that the WD patient's immune system attacks copper accumulated hepatocytes. Here we describe that the frequency and absolute number of V\u03b124+V\u03b211+ natural killer T (NKT) cells were significantly increased in 3 cases of WD, whereas those of CD3+CD161+ NKT cells were within the normal range. Patients no. 1 and 2 had a presymptomatic form of WD. Their tissue specimens showed pathological changes of mild degeneration of hepatocytes with a few infiltrating mononuclear cells and a low degree of fatty change. Patient no. 3 displayed fulminant hepatitis with Coombs-negative haemolytic anaemia. The tissue specimens of patient no. 3 showed macronodular cirrhosis with thick fibrosis, inflammatory infiltrates and spotty necrosis. Human V\u03b124+V\u03b211+ NKT cells are almost equal to CD1d-restricted NKT cells. Therefore we investigated CD1d-restricted NKT cells in the LEC rat as an animal model of WD. In LEC rats before hepatitis onset, the number and phenotype of liver NKT cells were normal. At about 4\u2003months of age all LEC rats developed acute hepatitis accompanied by acute jaundice, and CD161high NKT cells developed in their livers. CD161high\u03b1\u03b2TCRbright NKT cells developed in some of them. Their hepatitis was severe. CD161high\u03b1\u03b2TCRbright NKT cells expressed an invariant rat V\u03b114-J\u03b1281 chain, which is CD1d-restricted. Furthermore, liver lymphocytes in the acute jaundiced LEC rats with CD161high\u03b1\u03b2TCRbright NKT cells had significant and CD1d-specific cytotoxic activity.<\/jats:p>","DOI":"10.1111\/j.1365-2249.2005.02664.x","type":"journal-article","created":{"date-parts":[[2004,12,14]],"date-time":"2004-12-14T15:38:59Z","timestamp":1103038739000},"page":"144-151","source":"Crossref","is-referenced-by-count":8,"title":["Contribution of Va24+Vb11+ natural killer T cells in Wilsonian hepatitis"],"prefix":"10.1093","volume":"139","author":[{"given":"M","family":"Kinebuchi","sequence":"first","affiliation":[{"name":"Department of Pathology, School of Medicine, Fujita Health University, Toyoake"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A","family":"Matsuura","sequence":"additional","affiliation":[{"name":"Department of Pathology, School of Medicine, Fujita Health University, Toyoake"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K","family":"Ohya","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Aomori Central Hospital, Aomori"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W","family":"Abo","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Aomori Central Hospital, Aomori"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J","family":"Kitazawa","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Hirosaki City Hospital, Hirosaki, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2004,12,14]]},"reference":[{"key":"2022010900425092500_b1","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1038\/ng1293-327","article-title":"The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene","volume":"5","author":"Bull","year":"1993","journal-title":"Nat Genet"},{"key":"2022010900425092500_b2","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":"Nat Genet"},{"key":"2022010900425092500_b3","doi-asserted-by":"crossref","first-page":"1927","DOI":"10.1093\/hmg\/9.13.1927","article-title":"Copper-dependent trafficking of Wilson disease mutant ATP7B proteins","volume":"9","author":"Forbes","year":"2000","journal-title":"Hum Mol Genet"},{"key":"2022010900425092500_b4","doi-asserted-by":"crossref","first-page":"33279","DOI":"10.1074\/jbc.272.52.33279","article-title":"Expression, purification, and metal binding properties of the N-terminal domain from the wilson disease putative copper-transporting ATPase (ATP7B)","volume":"272","author":"DiDonato","year":"1997","journal-title":"J Biol Chem"},{"key":"2022010900425092500_b5","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1007\/s00431-001-0865-9","article-title":"Presymptomatic diagnosis of Wilson disease associated with a novel mutation of the ATP7B gene","volume":"161","author":"Ohya","year":"2002","journal-title":"Eur J Pediatr"},{"key":"2022010900425092500_b6","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1002\/eji.1830170107","article-title":"Large granular lymphocytes or \u2018pit cells\u2019 from rat liver: isolation, ultrastructural characterization and natural killer activity","volume":"17","author":"Bouwens","year":"1987","journal-title":"Eur J Immuno"},{"key":"2022010900425092500_b7","doi-asserted-by":"crossref","first-page":"3140","DOI":"10.4049\/jimmunol.164.6.3140","article-title":"NKT Cells in the Rat: Organ-specific distribution of NK T cells expressing distinct V14 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Cells","volume":"162","author":"Eberl","year":"1999","journal-title":"J Immunol"},{"key":"2022010900425092500_b11","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's disease","volume":"356","author":"Gu","year":"2000","journal-title":"Lancet"},{"key":"2022010900425092500_b12","first-page":"200","article-title":"The LEC rat: a model for human hepatitis, liver cancer, and much more","volume":"144","author":"Mori","year":"1994","journal-title":"Am J Pathol"},{"key":"2022010900425092500_b13","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1007\/s00439-003-1045-y","article-title":"A comparison of the mutation spectra of Menkes disease and Wilson disease","volume":"114","author":"His","year":"2004","journal-title":"Hum Genet"},{"key":"2022010900425092500_b14","doi-asserted-by":"crossref","first-page":"3294","DOI":"10.1073\/pnas.051604498","article-title":"Human 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