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Minihepcidins improve ineffective erythropoiesis and splenomegaly in a new mouse model of adult ß-thalassemia major.
Casu, Carla; Chessa, Roberta; Liu, Alison; Gupta, Ritama; Drakesmith, Hal; Fleming, Robert; Ginzburg, Yelena Z; MacDonald, Brian; Rivella, Stefano.
Afiliación
  • Casu C; Department of Pediatrics, Division of Hematology, The Children's Hospital of Philadelphia (CHOP), Philadelphia, PA, USA.
  • Chessa R; Department of Pediatrics, Division of Hematology, The Children's Hospital of Philadelphia (CHOP), Philadelphia, PA, USA.
  • Liu A; Department of Pediatrics, Division of Hematology, The Children's Hospital of Philadelphia (CHOP), Philadelphia, PA, USA.
  • Gupta R; Department of Pediatrics, Division of Hematology, The Children's Hospital of Philadelphia (CHOP), Philadelphia, PA, USA.
  • Drakesmith H; MRC Human Immunology Unit, MRC Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, UK.
  • Fleming R; Department of Pediatrics, Saint Louis University School of Medicine, St. Louis, MO, USA.
  • Ginzburg YZ; Division of Hematology and Medical Oncology, Tisch Cancer Center, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
  • MacDonald B; Merganser Biotech Inc. King of Prussia, PA, USA.
  • Rivella S; Department of Pediatrics, Division of Hematology, The Children's Hospital of Philadelphia (CHOP), Philadelphia, PA, USA rivellas@email.chop.edu.
Haematologica ; 105(7): 1835-1844, 2020 07.
Article en En | MEDLINE | ID: mdl-31582543
Minihepcidins are hepcidin agonists that have been previously shown to reverse iron overload and improve erythropoiesis in mice affected by non-transfusion-dependent thalassemia. Given the extreme anemia that occurred with the previous model of transfusion-dependent thalassemia, that model was inadequate for investigating whether minihepcidins can improve red blood cell quality, lifespan and ineffective erythropoiesis. To overcome this limitation, we generated a new murine model of transfusion-dependent thalassemia with severe anemia and splenomegaly, but sufficient red cells and hemoglobin production to test the effect of minihepcidins. Furthermore, this new model demonstrates cardiac iron overload for the first time. In the absence of transfusions, minihepcidins improved red blood cell morphology and lifespan as well as ineffective erythropoiesis. Administration of a minihepcidin in combination with chronic red blood cell transfusion further improved the ineffective erythropoiesis and splenomegaly and reversed cardiac iron overload. These studies indicate that drugs such as minihepcidins have therapeutic potential for patients with transfusion-dependent thalassemia.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Esplenomegalia / Talasemia beta / Sobrecarga de Hierro / Hepcidinas Tipo de estudio: Etiology_studies Límite: Animals Idioma: En Revista: Haematologica Año: 2020 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Italia
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Esplenomegalia / Talasemia beta / Sobrecarga de Hierro / Hepcidinas Tipo de estudio: Etiology_studies Límite: Animals Idioma: En Revista: Haematologica Año: 2020 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Italia