The hereditary hemochromatosis protein HFE promotes the expression of hepcidin, a circulating hormone produced by the liver that inhibits dietary iron absorption and macrophage iron release. HFE mutations are associated with impaired hepatic bone morphogenetic protein (BMP)/SMAD signaling for hepcidin production. TMPRSS6, a transmembrane serine protease mutated in iron-refractory iron deficienc...

BACKGROUND Liver iron deposits are frequent in viral C cirrhotic patients but their role is not well defined. AIMS To investigate the effect of liver iron excess on the prevalence of hepatocellular carcinoma (HCC) in patients with viral C cirrhosis. METHODS Hepatic iron was evaluated retrospectively using a semiquantitative method in liver biopsies of 104 viral C cirrhotic patients, 48 with...

The iron-regulatory hormone hepcidin is a 25-amino acid peptide that is synthesized in hepatocytes. Hepcidin binds to the cellular iron export channel ferroportin and causes its internalization and degradation and thereby decreases iron efflux from iron exporting tissues into plasma. By this mechanism, hepcidin inhibits dietary iron absorption, the efflux of recycled iron from splenic and hepat...

background: iron is an essential mineral for normal cellular physiology but its ‎overload can lead to cell injury. for many years, deferoxamine injection has been used ‎as an iron chelator for treatment of iron overload. the aim of this study is to compare ‎oral deferoxamine, activated charcoal, and vitamin c, as an absorbent factor of fe, in ‎changing the serum level of iron in iron overload r...

The p.M172K TFR2 mutation was identified in two Italian siblings aged 32 and 40 years old with primary iron overload. The two patients showed a severe increase in serum iron indices. From the age of 25, the male sib also revealed abnormal levels of hepatic enzymes, presumably in relation to iron induced liver damage. Clinical findings seem to evidence that type 3 hemochromatosis can be more ser...

β-thalassemia (βT) is a genetic blood disorder causing profound and life threatening anemia. Current clinical management of βT is a lifelong dependence on regular blood transfusions, a consequence of which is systemic iron overload leading to acute heart failure. Recent developments in gene and chelation therapy give hope of better prognosis for patients, but successful translation to clinical ...

D ESFERRIOXAMINE (DF) is currently the most eflective chelator available for the treatment of iron overload in man.’2 Intramuscular injections reduce the rate of iron accumulation and have been shown to arrest the progress of hepatic fibrosis in children with transfusion-dependent thalassemia.3 However, used in this fashion, the drug removes a limited amount of iron, and there has been extensiv...

BACKGROUND The 70% methanol extract of Terminalia chebula Retz. fruit (TCME) was investigated for its in vitro iron chelating property and in vivo ameliorating effect on hepatic injury of iron overloaded mice. METHODS The effect of fruit extract on Fe2+-ferrozine complex formation and Fe2+ mediated pUC-18 DNA breakdown was studied in order to find the in vitro iron chelating activity. Thirty-...

The accumulation of iron (Fe) in the liver is a serious complication of thalassemia and other ironloading anemias requiring long-term blood transfusions. Such patients are directed to suffer death unless they are treated with Fe chelating agents. Desferrioxamine (DFO), deferiprone (L1) and desferasirox (ICL-670) are clinically approved iron chelators used to treat secondary iron overload, but t...

isolated rat hepatocytes in culture were incubated with different concentrations of iron-sorbitol (50, 100, 150, and 200 µm) to assess the changes in reactive oxygen species (ros) and lipid peroxidation leading to apoptotic hepatocyte cell death. the viability of hepatocytes was declined depending on the iron concentration. one hour incubation of the cells with 100 µm iron resulted in decreased...