{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T06:47:59Z","timestamp":1777099679239,"version":"3.51.4"},"reference-count":46,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,8,24]],"date-time":"2006-08-24T00:00:00Z","timestamp":1156377600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2006,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p> <jats:italic>\u03b2<\/jats:italic>\u2010Thalassaemia represents a group of diseases, in which ineffective erythropoiesis is accompanied by iron overload. In a mouse model of <jats:italic>\u03b2<\/jats:italic>\u2010thalassaemia, we observed that the liver expressed relatively low levels of hepcidin, which is a key factor in the regulation of iron absorption by the gut and of iron recycling by the reticuloendothelial system. It was hypothesised that, despite the overt iron overload, a putative plasma factor found in <jats:italic>\u03b2<\/jats:italic>\u2010thalassaemia might suppress liver hepcidin expression. Sera from <jats:italic>\u03b2<\/jats:italic>\u2010thalassaemia and haemochromatosis (C282Y mutation) patients were compared with those of healthy individuals regarding their capacity to induce changes the expression of key genes of iron metabolism in human HepG2 hepatoma cells. Sera from <jats:italic>\u03b2<\/jats:italic>\u2010thalassaemia major patients induced a major decrease in hepcidin (<jats:italic>HAMP<\/jats:italic>) and lipocalin2 (oncogene 24p3) (<jats:italic>LCN2<\/jats:italic>) expression, as well as a moderate decrease in haemojuvelin (<jats:italic>HFE2<\/jats:italic>) expression, compared with sera from healthy individuals. A significant correlation was found between the degree of downregulation of <jats:italic>HAMP<\/jats:italic> and <jats:italic>HFE2<\/jats:italic> induced by <jats:italic>\u03b2<\/jats:italic>\u2010thalassaemia major sera (<jats:italic>r<\/jats:italic>\u2003=\u20030\u00b7852, <jats:italic>P<\/jats:italic>\u2003&lt;\u20030\u00b70009). Decreased <jats:italic>HAMP<\/jats:italic> expression was also found in HepG2 cells treated with sera from <jats:italic>\u03b2<\/jats:italic>\u2010thalassaemia intermedia patients. In contrast, the majority of sera from hereditary haemochromatosis patients induced an increase in <jats:italic>HAMP<\/jats:italic> expression, which correlated with transferrin (Tf) saturation (<jats:italic>r<\/jats:italic>\u2003=\u20030\u00b7765, <jats:italic>P<\/jats:italic>\u2003&lt;\u20030\u00b70099). Our results suggest that, in <jats:italic>\u03b2<\/jats:italic>\u2010thalassaemia, serum factors might override the potential effect of iron overload on <jats:italic>HAMP<\/jats:italic> expression, thereby providing an explanation for the failure to arrest excessive intestinal iron absorption in these patients.<\/jats:p>","DOI":"10.1111\/j.1365-2141.2006.06258.x","type":"journal-article","created":{"date-parts":[[2006,8,29]],"date-time":"2006-08-29T18:15:28Z","timestamp":1156875328000},"page":"129-138","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":58,"title":["Downregulation of hepcidin and haemojuvelin expression in the hepatocyte cell\u2010line HepG2 induced by thalassaemic 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