Ineffective Erythropoiesis in β-Thalassemia
In humans, β-thalassemia dyserythropoiesis is characterized by expansion of early erythroid precursors and erythroid progenitors and then ineffective erythropoiesis. This ineffective erythropoiesis is defined as a suboptimal production of mature erythrocytes originating from a proliferating pool of...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | The Scientific World Journal |
| Online Access: | http://dx.doi.org/10.1155/2013/394295 |
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| author | Jean-Antoine Ribeil Jean-Benoit Arlet Michael Dussiot Ivan Cruz Moura Geneviève Courtois Olivier Hermine |
| author_facet | Jean-Antoine Ribeil Jean-Benoit Arlet Michael Dussiot Ivan Cruz Moura Geneviève Courtois Olivier Hermine |
| author_sort | Jean-Antoine Ribeil |
| collection | DOAJ |
| description | In humans, β-thalassemia dyserythropoiesis is characterized by expansion of early erythroid precursors and erythroid progenitors and then ineffective erythropoiesis. This ineffective erythropoiesis is defined as a suboptimal production of mature erythrocytes originating from a proliferating pool of immature erythroblasts. It is characterized by (1) accelerated erythroid differentiation, (2) maturation blockade at the polychromatophilic stage, and (3) death of erythroid precursors. Despite extensive knowledge of molecular defects causing β-thalassemia, less is known about the mechanisms responsible for ineffective erythropoiesis. In this paper, we will focus on the underlying mechanisms leading to premature death of thalassemic erythroid precursors in the bone marrow. |
| format | Article |
| id | doaj-art-76f900d08c044fda95f25a901e894d0e |
| institution | Kabale University |
| issn | 1537-744X |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | The Scientific World Journal |
| spelling | doaj-art-76f900d08c044fda95f25a901e894d0e2025-02-03T00:59:58ZengWileyThe Scientific World Journal1537-744X2013-01-01201310.1155/2013/394295394295Ineffective Erythropoiesis in β-ThalassemiaJean-Antoine Ribeil0Jean-Benoit Arlet1Michael Dussiot2Ivan Cruz Moura3Geneviève Courtois4Olivier Hermine5Centre National de la Recherche Scientifique-Unité Mixte de Recherche 8147, Université Paris V, René Descartes, Hôpital Necker, Paris, FranceCentre National de la Recherche Scientifique-Unité Mixte de Recherche 8147, Université Paris V, René Descartes, Hôpital Necker, Paris, FranceCentre National de la Recherche Scientifique-Unité Mixte de Recherche 8147, Université Paris V, René Descartes, Hôpital Necker, Paris, FranceFondation Imagine, Institut des Maladies Génétiques, Faculté de Médecine Paris Descartes, Sorbonne Paris-Cité et Assistance Publique—Hôpitaux de Paris, Hôpital Necker, Paris, FranceCentre National de la Recherche Scientifique-Unité Mixte de Recherche 8147, Université Paris V, René Descartes, Hôpital Necker, Paris, FranceCentre National de la Recherche Scientifique-Unité Mixte de Recherche 8147, Université Paris V, René Descartes, Hôpital Necker, Paris, FranceIn humans, β-thalassemia dyserythropoiesis is characterized by expansion of early erythroid precursors and erythroid progenitors and then ineffective erythropoiesis. This ineffective erythropoiesis is defined as a suboptimal production of mature erythrocytes originating from a proliferating pool of immature erythroblasts. It is characterized by (1) accelerated erythroid differentiation, (2) maturation blockade at the polychromatophilic stage, and (3) death of erythroid precursors. Despite extensive knowledge of molecular defects causing β-thalassemia, less is known about the mechanisms responsible for ineffective erythropoiesis. In this paper, we will focus on the underlying mechanisms leading to premature death of thalassemic erythroid precursors in the bone marrow.http://dx.doi.org/10.1155/2013/394295 |
| spellingShingle | Jean-Antoine Ribeil Jean-Benoit Arlet Michael Dussiot Ivan Cruz Moura Geneviève Courtois Olivier Hermine Ineffective Erythropoiesis in β-Thalassemia The Scientific World Journal |
| title | Ineffective Erythropoiesis in β-Thalassemia |
| title_full | Ineffective Erythropoiesis in β-Thalassemia |
| title_fullStr | Ineffective Erythropoiesis in β-Thalassemia |
| title_full_unstemmed | Ineffective Erythropoiesis in β-Thalassemia |
| title_short | Ineffective Erythropoiesis in β-Thalassemia |
| title_sort | ineffective erythropoiesis in β thalassemia |
| url | http://dx.doi.org/10.1155/2013/394295 |
| work_keys_str_mv | AT jeanantoineribeil ineffectiveerythropoiesisinbthalassemia AT jeanbenoitarlet ineffectiveerythropoiesisinbthalassemia AT michaeldussiot ineffectiveerythropoiesisinbthalassemia AT ivancruzmoura ineffectiveerythropoiesisinbthalassemia AT genevievecourtois ineffectiveerythropoiesisinbthalassemia AT olivierhermine ineffectiveerythropoiesisinbthalassemia |