{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T22:13:59Z","timestamp":1787004839491,"version":"build-2736575974"},"reference-count":0,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":5325,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2001,2]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>\n                      Porphyria cutanea tarda (PCT), a liver disease with skin lesions caused by excess liver production of uroporphyrin (URO), is associated with consumption of alcoholic beverages or estrogens, and moderate iron overload. Recently, it has been shown that many PCT patients carry mutations in the\n                      <jats:italic toggle=\"yes\">HFE<\/jats:italic>\n                      gene, which is responsible for hereditary hemochromatosis. Mice homozygous for either the null mutation in the\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      gene or the C282Y missense mutation rapidly accumulate hepatic parenchymal iron similar to patients with hemochromatosis. Here we investigated whether disruption of the murine\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      gene would result in hepatic uroporphyria. Mice homozygous for the\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      \u2013null mutation accumulated high levels of hepatic URO when fed 5\u2013aminolevulinate (ALA).\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      (+\/-) mice also accumulated hepatic URO when fed ALA, but at a much slower rate. The amount of accumulated URO in the null mutant mice was similar to that in wild\u2013type mice treated with iron carbonyl in the diet, or injected with iron dextran. Iron in both wild\u2013type and\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      (+\/-) mice was mostly in Kupffer cells. In contrast,\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      (-\/-) mice had considerable parenchymal iron deposition as well, in a pattern similar to that observed in wild\u2013type mice treated with iron carbonyl. URO accumulation was accompanied by 84% and 33% decreases in hepatic uroporphyrinogen decarboxylase activities in\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      (-\/-) and\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      (+\/-) mice, respectively. No increases in CYP1A2 or other cytochrome P450s were detected in the\n                      <jats:italic toggle=\"yes\">Hfe<\/jats:italic>\n                      \u2013null mutant mice. We conclude that this experimental model of uroporphyria is a valid model for further investigations into the mechanism of PCT.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1053\/jhep.2001.21409","type":"journal-article","created":{"date-parts":[[2002,8,24]],"date-time":"2002-08-24T23:46:10Z","timestamp":1030232770000},"page":"406-412","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":20,"title":["Uroporphyria In Hfe Mutant Mice Given 5\u2013Aminolevulinate: A New Model of Fe\u2013Mediated Porphyria Cutanea Tarda"],"prefix":"10.1097","volume":"33","author":[{"given":"Peter R.","family":"Sinclair","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Gorman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi S.","family":"Walton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William J.","family":"Bement","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline F.","family":"Sinclair","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glenn S.","family":"Gerhard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliana G.","family":"Szakacs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nancy C.","family":"Andrews","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanne E.","family":"Levy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","container-title":["Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1053%2Fjhep.2001.21409","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01515467-200102000-00012","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,1]],"date-time":"2024-12-01T06:27:41Z","timestamp":1733034461000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01515467-200102000-00012"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,2]]},"references-count":0,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2001]]}},"URL":"https:\/\/doi.org\/10.1053\/jhep.2001.21409","relation":{},"ISSN":["0270-9139"],"issn-type":[{"value":"0270-9139","type":"print"}],"subject":[],"published":{"date-parts":[[2001,2]]}}}