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• W4289202346 endingPage "1336" @default.
• W4289202346 startingPage "1324" @default.
• W4289202346 abstract "β-thalassemia is a genetic disorder caused by mutations in the β-globin gene, and characterized by anemia, ineffective erythropoiesis and iron overload. Patients affected by the most severe transfusion-dependent form of the disease (TDT) require lifelong blood transfusions and iron chelation therapy, a symptomatic treatment associated with several complications. Other therapeutic opportunities are available, but none is fully effective and/or applicable to all patients, calling for the identification of novel strategies. Transferrin receptor 2 (TFR2) balances red blood cells production according to iron availability, being an activator of the iron-regulatory hormone hepcidin in the liver and a modulator of erythropoietin signaling in erythroid cells. Selective Tfr2 deletion in the BM improves anemia and iron-overload in non-TDT mice, both as a monotherapy and, even more strikingly, in combination with iron-restricting approaches. However, whether Tfr2 targeting might represent a therapeutic option for TDT has never been investigated so far. Here, we prove that BM Tfr2 deletion improves anemia, erythrocytes morphology and ineffective erythropoiesis in the Hbbth1/th2 murine model of TDT. This effect is associated with a decrease in the expression of α-globin, which partially corrects the unbalance with β-globin chains and limits the precipitation of misfolded hemoglobin, and with a decrease in the activation of unfolded protein response. Remarkably, BM Tfr2 deletion is also sufficient to avoid long-term blood transfusions required for survival of Hbbth1/th2 animals, preventing mortality due to chronic anemia and reducing transfusion-associated complications, such as progressive iron-loading. Altogether, TFR2 targeting might represent a promising therapeutic option also for TDT." @default.
• W4289202346 created "2022-08-01" @default.
• W4289202346 creator A5005334724 @default.
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• W4289202346 date "2022-08-10" @default.
• W4289202346 modified "2023-10-17" @default.
• W4289202346 title "<i>Transferrin receptor 2 (Tfr2)</i> genetic deletion makes transfusion‐independent a murine model of transfusion‐dependent β‐thalassemia" @default.
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• W4289202346 doi "https://doi.org/10.1002/ajh.26673" @default.
• W4289202346 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/36071579" @default.
• W4289202346 hasPublicationYear "2022" @default.
• W4289202346 type Work @default.

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