Ineffective Erythropoiesis in β-Thalassemia

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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Main Authors: Ribeil, Jean-Antoine, Arlet, Jean-Benoit, Dussiot, Michael, Cruz Moura, Ivan, Courtois, Geneviève, Hermine, Olivier
Format: Online
Language:English
Published: Hindawi Publishing Corporation 2013
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3628659/
id pubmed-3628659
recordtype oai_dc
spelling pubmed-36286592013-04-19 Ineffective Erythropoiesis in β-Thalassemia Ribeil, Jean-Antoine Arlet, Jean-Benoit Dussiot, Michael Cruz Moura, Ivan Courtois, Geneviève Hermine, Olivier Review Article 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. Hindawi Publishing Corporation 2013-03-28 /pmc/articles/PMC3628659/ /pubmed/23606813 http://dx.doi.org/10.1155/2013/394295 Text en Copyright © 2013 Jean-Antoine Ribeil et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
repository_type Open Access Journal
institution_category Foreign Institution
institution US National Center for Biotechnology Information
building NCBI PubMed
collection Online Access
language English
format Online
author Ribeil, Jean-Antoine
Arlet, Jean-Benoit
Dussiot, Michael
Cruz Moura, Ivan
Courtois, Geneviève
Hermine, Olivier
spellingShingle Ribeil, Jean-Antoine
Arlet, Jean-Benoit
Dussiot, Michael
Cruz Moura, Ivan
Courtois, Geneviève
Hermine, Olivier
Ineffective Erythropoiesis in β-Thalassemia
author_facet Ribeil, Jean-Antoine
Arlet, Jean-Benoit
Dussiot, Michael
Cruz Moura, Ivan
Courtois, Geneviève
Hermine, Olivier
author_sort Ribeil, Jean-Antoine
title Ineffective Erythropoiesis in β-Thalassemia
title_short 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_sort ineffective erythropoiesis in β-thalassemia
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.
publisher Hindawi Publishing Corporation
publishDate 2013
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3628659/
_version_ 1611970701403619328

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