Combination of Tmprss6- ASO and the iron chelator deferiprone improves erythropoiesis and reduces iron overload in a mouse model of beta-thalassemia intermedia.
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چکیده
منابع مشابه
Combination therapy with a Tmprss6 RNAi-therapeutic and the oral iron chelator deferiprone additively diminishes secondary iron overload in a mouse model of β-thalassemia intermedia
β-thalassemias result from diminished β-globin synthesis and are associated with ineffective erythropoiesis and secondary iron overload caused by inappropriately low levels of the iron regulatory hormone hepcidin. The serine protease TMPRSS6 attenuates hepcidin production in response to iron stores. Hepcidin induction reduces iron overload and mitigates anemia in murine models of β-thalassemia ...
متن کاملDeletion of TMPRSS6 attenuates the phenotype in a mouse model of β-thalassemia.
Inappropriately low expression of the key iron regulator hepcidin (HAMP) causes iron overload in untransfused patients affected by β-thalassemia intermedia and Hamp modulation provides improvement of the thalassemic phenotype of the Hbb(th3/+) mouse. HAMP expression is activated by iron through the bone morphogenetic protein (BMP)-son of mothers against decapentaplegic signaling pathway and inh...
متن کاملRED CELLS, IRON, AND ERYTHROPOIESIS Deletion of TMPRSS6 attenuates the phenotype in a mouse model of -thalassemia
Inappropriately low expression of the key iron regulator hepcidin (HAMP) causes iron overload in untransfused patients affected by -thalassemia intermedia and Hamp modulation provides improvement of the thalassemic phenotype of the Hbbth3/ mouse. HAMP expression is activated by iron through the bone morphogenetic protein (BMP)–son of mothers against decapentaplegic signaling pathway and inhibit...
متن کاملIron chelator drugs in thalassemia,a review article
Abstract Thalassemia syndromes are the most common inherited hemoglobinopathies in the world characterized by various degrees of defective production of the alpha or beta globin chains. Impairment in alpha chain or beta chain synthesis leads to alpha thalassemia and beta thalassemia syndromes respectively. Iran is one of the countries located on the thalassemia belt therefore this disease is ...
متن کاملComparison of Magnetic Resonance Imaging T2 Results in Beta-Thalassemia Patients Treated by Deferasirox or Combination of Deferoxamine and Deferiprone
Background: Iron extra load is an anticipated and lethal consequence of chronic blood transfusion in major beta-thalassemia patients; therefore it is necessary to use an efficient iron chelator drug to stimulate the evacuation of the surplus iron from the body. This trial was performed to compare myocardial and hepatic magnetic resonance imaging T2 (MRI T2*) results of beta-thalassemia patients...
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ورودعنوان ژورنال:
- Haematologica
دوره 101 1 شماره
صفحات -
تاریخ انتشار 2016