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Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease

Introduction. Sickle cell disease (SCD) is characterized by hemoglobin S homozygosity, leading to hemolysis and vasoocclusion. The hemolysis releases arginase I, an enzyme that decreases the bioavailability of nitric oxide, worsening the symptoms. The different SCD haplotypes are related to clinical...

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Autores principales: Moreira, J. A., Machado, R. P. G., Laurentino, M. R., Lemes, Romelia Pinheiro Gonçalves, Barbosa, M. C., Santos, T. E., Bandeira, I. C. J., Martins, A. M. C.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4870341/
https://www.ncbi.nlm.nih.gov/pubmed/27274608
http://dx.doi.org/10.1155/2016/9172726
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author Moreira, J. A.
Machado, R. P. G.
Laurentino, M. R.
Lemes, Romelia Pinheiro Gonçalves
Barbosa, M. C.
Santos, T. E.
Bandeira, I. C. J.
Martins, A. M. C.
author_facet Moreira, J. A.
Machado, R. P. G.
Laurentino, M. R.
Lemes, Romelia Pinheiro Gonçalves
Barbosa, M. C.
Santos, T. E.
Bandeira, I. C. J.
Martins, A. M. C.
author_sort Moreira, J. A.
collection PubMed
description Introduction. Sickle cell disease (SCD) is characterized by hemoglobin S homozygosity, leading to hemolysis and vasoocclusion. The hemolysis releases arginase I, an enzyme that decreases the bioavailability of nitric oxide, worsening the symptoms. The different SCD haplotypes are related to clinical symptoms and varied hemoglobin F (HbF) concentration. The aim of this study was to evaluate the impact of the βS gene haplotypes and HbF concentration on arginase I levels in SCD patients. Methods. Fifty SCD adult patients were enrolled in the study and 20 blood donors composed the control group. Arginase I was measured by ELISA. The βS haplotypes were identified by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Statistical analyses were performed with GraphPad Prism program and the significance level was p < 0.05. Results. Significant increase was observed in the arginase I levels in SCD patients compared to the control group (p < 0.0001). The comparison between the levels of arginase I in three haplotypes groups showed a difference between the Bantu/Bantu × Bantu/Benin groups; Bantu/Bantu × Benin/Benin, independent of HU dosage. An inverse correlation with the arginase I levels and HbF concentration was observed. Conclusion. The results support the hypothesis that arginase I is associated with HbF concentration, also measured indirectly by the association with haplotypes.
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spelling pubmed-48703412016-06-05 Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease Moreira, J. A. Machado, R. P. G. Laurentino, M. R. Lemes, Romelia Pinheiro Gonçalves Barbosa, M. C. Santos, T. E. Bandeira, I. C. J. Martins, A. M. C. Dis Markers Research Article Introduction. Sickle cell disease (SCD) is characterized by hemoglobin S homozygosity, leading to hemolysis and vasoocclusion. The hemolysis releases arginase I, an enzyme that decreases the bioavailability of nitric oxide, worsening the symptoms. The different SCD haplotypes are related to clinical symptoms and varied hemoglobin F (HbF) concentration. The aim of this study was to evaluate the impact of the βS gene haplotypes and HbF concentration on arginase I levels in SCD patients. Methods. Fifty SCD adult patients were enrolled in the study and 20 blood donors composed the control group. Arginase I was measured by ELISA. The βS haplotypes were identified by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Statistical analyses were performed with GraphPad Prism program and the significance level was p < 0.05. Results. Significant increase was observed in the arginase I levels in SCD patients compared to the control group (p < 0.0001). The comparison between the levels of arginase I in three haplotypes groups showed a difference between the Bantu/Bantu × Bantu/Benin groups; Bantu/Bantu × Benin/Benin, independent of HU dosage. An inverse correlation with the arginase I levels and HbF concentration was observed. Conclusion. The results support the hypothesis that arginase I is associated with HbF concentration, also measured indirectly by the association with haplotypes. Hindawi Publishing Corporation 2016 2016-05-04 /pmc/articles/PMC4870341/ /pubmed/27274608 http://dx.doi.org/10.1155/2016/9172726 Text en Copyright © 2016 J. A. Moreira et al. https://creativecommons.org/licenses/by/4.0/ 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.
spellingShingle Research Article
Moreira, J. A.
Machado, R. P. G.
Laurentino, M. R.
Lemes, Romelia Pinheiro Gonçalves
Barbosa, M. C.
Santos, T. E.
Bandeira, I. C. J.
Martins, A. M. C.
Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease
title Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease
title_full Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease
title_fullStr Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease
title_full_unstemmed Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease
title_short Influence of βS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease
title_sort influence of βs-globin haplotypes and hydroxyurea on arginase i levels in sickle cell disease
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4870341/
https://www.ncbi.nlm.nih.gov/pubmed/27274608
http://dx.doi.org/10.1155/2016/9172726
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