Decreased hepcidin expression in murine β-thalassemia is associated with suppression of Bmp/Smad signaling

NL Parrow, S Gardenghi, P Ramos… - Blood, The Journal …, 2012 - ashpublications.org
NL Parrow, S Gardenghi, P Ramos, C Casu, RW Grady, ER Anderson, YM Shah, H Li…
Blood, The Journal of the American Society of Hematology, 2012ashpublications.org
Decreased hepcidin expression in murine ß-thalassemia is associated with suppression of
Bmp/Smad signaling-thalassemia is a genetic disorder of hemoglobin production
characterized by ineffective erythropoiesis and anemia. 1 Iron overload, a major source of
morbidity, results from inappropriately low expression of the gene encoding hepcidin
(Hamp1). 1 Hamp1 controls plasma iron concentration by facilitating the degradation of the
ironeffluxproteinferroportin. 2 Inhealthyindividuals, hepcidinistranscriptionally responsive to …
Decreased hepcidin expression in murine ß-thalassemia is associated with suppression of Bmp/Smad signaling
-thalassemia is a genetic disorder of hemoglobin production characterized by ineffective erythropoiesis and anemia. 1 Iron overload, a major source of morbidity, results from inappropriately low expression of the gene encoding hepcidin (Hamp1). 1 Hamp1 controls plasma iron concentration by facilitating the degradation of the ironeffluxproteinferroportin. 2 Inhealthyindividuals, hepcidinistranscriptionally responsive to iron via bone morphogenetic protein (Bmp)/Smad pathway-mediated phosphorylation of Smad 1, 5, 8. 3 Phosphorylated Smad 1, 5, 8 is an essential component of the transcriptional complex that induces Hamp1 expression in response to iron. 4 We investigated the
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