Physiological and Aberrant γ-Globin Transcription During Development
The expression of the fetal Gγ- and Aγ-globin genes in normal development is confined to the fetal period, where two γ-globin chains assemble with two α-globin chains to form α2γ2 tetramers (HbF). HbF sustains oxygen delivery to tissues until birth, when β-globin replaces γ-globin, leading to the fo...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2021-04-01
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| Series: | Frontiers in Cell and Developmental Biology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fcell.2021.640060/full |
| _version_ | 1819170627833036800 |
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| author | Gloria Barbarani Agata Labedz Sarah Stucchi Alessia Abbiati Antonella E. Ronchi |
| author_facet | Gloria Barbarani Agata Labedz Sarah Stucchi Alessia Abbiati Antonella E. Ronchi |
| author_sort | Gloria Barbarani |
| collection | DOAJ |
| description | The expression of the fetal Gγ- and Aγ-globin genes in normal development is confined to the fetal period, where two γ-globin chains assemble with two α-globin chains to form α2γ2 tetramers (HbF). HbF sustains oxygen delivery to tissues until birth, when β-globin replaces γ-globin, leading to the formation of α2β2 tetramers (HbA). However, in different benign and pathological conditions, HbF is expressed in adult cells, as it happens in the hereditary persistence of fetal hemoglobin, in anemias and in some leukemias. The molecular basis of γ-globin differential expression in the fetus and of its inappropriate activation in adult cells is largely unknown, although in recent years, a few transcription factors involved in this process have been identified. The recent discovery that fetal cells can persist to adulthood and contribute to disease raises the possibility that postnatal γ-globin expression could, in some cases, represent the signature of the fetal cellular origin. |
| first_indexed | 2024-12-22T19:38:24Z |
| format | Article |
| id | doaj.art-0cf3b9fad4784341913f5580d207f332 |
| institution | Directory Open Access Journal |
| issn | 2296-634X |
| language | English |
| last_indexed | 2024-12-22T19:38:24Z |
| publishDate | 2021-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cell and Developmental Biology |
| spelling | doaj.art-0cf3b9fad4784341913f5580d207f3322022-12-21T18:14:54ZengFrontiers Media S.A.Frontiers in Cell and Developmental Biology2296-634X2021-04-01910.3389/fcell.2021.640060640060Physiological and Aberrant γ-Globin Transcription During DevelopmentGloria BarbaraniAgata LabedzSarah StucchiAlessia AbbiatiAntonella E. RonchiThe expression of the fetal Gγ- and Aγ-globin genes in normal development is confined to the fetal period, where two γ-globin chains assemble with two α-globin chains to form α2γ2 tetramers (HbF). HbF sustains oxygen delivery to tissues until birth, when β-globin replaces γ-globin, leading to the formation of α2β2 tetramers (HbA). However, in different benign and pathological conditions, HbF is expressed in adult cells, as it happens in the hereditary persistence of fetal hemoglobin, in anemias and in some leukemias. The molecular basis of γ-globin differential expression in the fetus and of its inappropriate activation in adult cells is largely unknown, although in recent years, a few transcription factors involved in this process have been identified. The recent discovery that fetal cells can persist to adulthood and contribute to disease raises the possibility that postnatal γ-globin expression could, in some cases, represent the signature of the fetal cellular origin.https://www.frontiersin.org/articles/10.3389/fcell.2021.640060/fullglobin genestranscription factorshereditary persistence of fetal hemoglobinjuvenile myelomonocytic leukemiaerythropoiesis |
| spellingShingle | Gloria Barbarani Agata Labedz Sarah Stucchi Alessia Abbiati Antonella E. Ronchi Physiological and Aberrant γ-Globin Transcription During Development Frontiers in Cell and Developmental Biology globin genes transcription factors hereditary persistence of fetal hemoglobin juvenile myelomonocytic leukemia erythropoiesis |
| title | Physiological and Aberrant γ-Globin Transcription During Development |
| title_full | Physiological and Aberrant γ-Globin Transcription During Development |
| title_fullStr | Physiological and Aberrant γ-Globin Transcription During Development |
| title_full_unstemmed | Physiological and Aberrant γ-Globin Transcription During Development |
| title_short | Physiological and Aberrant γ-Globin Transcription During Development |
| title_sort | physiological and aberrant γ globin transcription during development |
| topic | globin genes transcription factors hereditary persistence of fetal hemoglobin juvenile myelomonocytic leukemia erythropoiesis |
| url | https://www.frontiersin.org/articles/10.3389/fcell.2021.640060/full |
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