Thalassemia

FOXO3-mTOR metabolic cooperation in the regulation of erythroid cell maturation and homeostasis

Dr. Rivella was part of a research group whose findings indicate a new course in which ineffective erythropoiesis develops. The study shows FOXO3 and mTOR transcription factors are center to a metabolic network in eryhtroblasts that control the balanced production and maturation of erytroid cells. Published in Journal of Hematology on October 2014.

Altered erythropoiesis and iron metabolism in carriers of thalassemia

Dr. Rivella was part of a team that proved thalassemia carriers have altered iron metabolism and erythropoiesis for the first time in literature. Published in the European Journal of Haematology on October 2014.

Reactivation of Developmentally Silenced Globin Genes by Forced Chromatin Looping

Dr. Rivella was part of a team that demonstrated forced chromatin looping can reactivate globin gene transcription in adult erythroblasts with silenced globin genes. Their findings may lead to a novel approach in controlling the balance of globin gene transcription.

Modified activin receptor IIB ligand trap mitigates ineffective erythropoiesis and disease complications in murine β-thalassemia

Dr. Rivella was part of a reserach group that found a possible novel therapeutic agent for thalassemia syndrome and other red cell disorders characterized by IE (ineffective erythropoiesis). 

Identification of erythroferrone as an erythroid regulator of iron metabolism

Dr. Rivella was part of a team that discovered a new hormone ERFE that mediates hepcidin suppression during stress erythropoisesis.

FGF-23 is a negative regulator of prenatal and postnatal erythropoiesis

Dr. Rivera was part of a team that discovered a possible novel role for FGF-23 in erythrocyte production and differentiation and also suggested elevated FGF-23 levels contribute to the pathogenesis of anemia in patients with CKD and CVD. Published in the Journal of Biological Chemistry on April 2014. 

Distinct roles for hepcidin and interleukin-6 in the recovery from anemia in mice injected with heat-killed Brucella abortus

Dr. Rivella was part of a research team that found evidence supporting the notion that interleukin 6 and hepcidin played distinct roles in modulating erythropoisesis in anemia of inflammation. Published in Blood on February 2014.

 

Magnetic Resonance Characterization of Hepatic Storage Iron in Transfusional Iron Overload

Dr. Sheth was part of a team that researched a new method in non-invasive MRI determination of the division of two different forms of iron in heptatic storage iron in iron overload disorders. Quantifying the two different types may help provide early warning of an increased risk of iron-induced toxicity and permit rapid monitoring of treatment.

Intestinal HIF2α promotes tissue-iron accumulation in disorders of iron overload with anemia

Dr. Rivella was part of a research group that demonstrated dysregulation of intestinal hypoxia and HIF2α signaling is critical for progressive iron overload in β-thalassemia and may be a new therapeutic target in several anemic iron-overload disorders. Published in PNAS on Novemeber 2013.

Macrophages support pathological erythropoiesis in polycythemia vera and β-thalassemia

Dr. Giardina was part of a reserach group that discovered the new pathophysiological mechanism (SEMA) found in polycythemia vera and β-thalassemia that will have important therapeutic implications in the near future. SEMA supports the signaling in RBC production and essential for erythroid expansion and differentiation. Published in Nature Medicine on April 2013.

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