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What influences Hb fetal production in adulthood?
Human hemoglobin genes are located in α and β globin gene clusters in chromosomes 16 and 11, respectively. Different types of hemoglobin are synthesized according to the stage of development with fetal hemoglobin (α(2)γ(2)) (Hb F) being the main hemoglobin in the fetal period. After birth, there is...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Associação Brasileira de Hematologia e Hemoterapia
2011
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3415736/ https://www.ncbi.nlm.nih.gov/pubmed/23049301 http://dx.doi.org/10.5581/1516-8484.20110059 |
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author | Carrocini, Gisele Cristine de Souza Zamaro, Paula Juliana Antoniazzo Bonini-Domingos, Claudia Regina |
author_facet | Carrocini, Gisele Cristine de Souza Zamaro, Paula Juliana Antoniazzo Bonini-Domingos, Claudia Regina |
author_sort | Carrocini, Gisele Cristine de Souza |
collection | PubMed |
description | Human hemoglobin genes are located in α and β globin gene clusters in chromosomes 16 and 11, respectively. Different types of hemoglobin are synthesized according to the stage of development with fetal hemoglobin (α(2)γ(2)) (Hb F) being the main hemoglobin in the fetal period. After birth, there is a reduction (to about 1%) in Hb F levels and adult hemoglobin, Hb A (2α(2)β(2)), increases to more than 96% of total hemoglobin. However, some genetic conditions whether linked to the β-globin gene cluster or not are associated with high Hb F levels in adults. Among those linked to β-globin are hereditary persistence of fetal hemoglobin, delta-beta thalassemia (δβ-Thalassemia) and the XmnI polymorphism (-158 C = T). Other polymorphisms not related to β-globin gene cluster are known to influence the γ-globin gene expression in adulthood. The most relevant polymorphisms that increase concentrations of Hb F are the HMIP locus on chromosome 6, the BCL11A locus on chromosome 2, the Xp22.2 region of the X chromosome and the 8q region on chromosome 8. Findings from our research group studying genetic factors involved in γ-globin gene regulation in adults without anemia in the northwestern region of São Paulo State showed that high Hb F levels are influenced by the presence of hereditary persistence of fetal hemoglobin mutations and the XmnI polymorphism, suggesting that both genetic alterations characterize the molecular basis of the evaluated population. |
format | Online Article Text |
id | pubmed-3415736 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Associação Brasileira de Hematologia e Hemoterapia |
record_format | MEDLINE/PubMed |
spelling | pubmed-34157362012-10-04 What influences Hb fetal production in adulthood? Carrocini, Gisele Cristine de Souza Zamaro, Paula Juliana Antoniazzo Bonini-Domingos, Claudia Regina Rev Bras Hematol Hemoter Review Article Human hemoglobin genes are located in α and β globin gene clusters in chromosomes 16 and 11, respectively. Different types of hemoglobin are synthesized according to the stage of development with fetal hemoglobin (α(2)γ(2)) (Hb F) being the main hemoglobin in the fetal period. After birth, there is a reduction (to about 1%) in Hb F levels and adult hemoglobin, Hb A (2α(2)β(2)), increases to more than 96% of total hemoglobin. However, some genetic conditions whether linked to the β-globin gene cluster or not are associated with high Hb F levels in adults. Among those linked to β-globin are hereditary persistence of fetal hemoglobin, delta-beta thalassemia (δβ-Thalassemia) and the XmnI polymorphism (-158 C = T). Other polymorphisms not related to β-globin gene cluster are known to influence the γ-globin gene expression in adulthood. The most relevant polymorphisms that increase concentrations of Hb F are the HMIP locus on chromosome 6, the BCL11A locus on chromosome 2, the Xp22.2 region of the X chromosome and the 8q region on chromosome 8. Findings from our research group studying genetic factors involved in γ-globin gene regulation in adults without anemia in the northwestern region of São Paulo State showed that high Hb F levels are influenced by the presence of hereditary persistence of fetal hemoglobin mutations and the XmnI polymorphism, suggesting that both genetic alterations characterize the molecular basis of the evaluated population. Associação Brasileira de Hematologia e Hemoterapia 2011 /pmc/articles/PMC3415736/ /pubmed/23049301 http://dx.doi.org/10.5581/1516-8484.20110059 Text en http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Carrocini, Gisele Cristine de Souza Zamaro, Paula Juliana Antoniazzo Bonini-Domingos, Claudia Regina What influences Hb fetal production in adulthood? |
title | What influences Hb fetal production in adulthood? |
title_full | What influences Hb fetal production in adulthood? |
title_fullStr | What influences Hb fetal production in adulthood? |
title_full_unstemmed | What influences Hb fetal production in adulthood? |
title_short | What influences Hb fetal production in adulthood? |
title_sort | what influences hb fetal production in adulthood? |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3415736/ https://www.ncbi.nlm.nih.gov/pubmed/23049301 http://dx.doi.org/10.5581/1516-8484.20110059 |
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