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Differences in response to fetal hemoglobin induction therapy in β-thalassemia and sickle cell disease

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dc.contributor.author Fathallah H.
dc.contributor.author Taher A.
dc.contributor.author Bazarbachi A.
dc.contributor.author Atweh G.F.
dc.contributor.editor
dc.date Apr-2009
dc.date.accessioned 2017-10-05T15:39:22Z
dc.date.available 2017-10-05T15:39:22Z
dc.date.issued 2009
dc.identifier 10.1016/j.bcmd.2009.02.006
dc.identifier.isbn
dc.identifier.issn 10799796
dc.identifier.uri http://hdl.handle.net/10938/16827
dc.description.abstract Inducers of fetal hemoglobin (HbF) have shown considerable promise in the treatment of sickle cell disease (SCD). However, the same agents have shown less clinical activity in β-thalassemia (β-Thal). To understand the basis of these differences in clinical effectiveness, we compared the effects of butyrate and hemin on the expression of the different globin genes in progenitors-derived erythroid cells from patients with β-Thal intermedia and SCD. Exposure to butyrate resulted in an augmentation of γ-globin mRNA levels in both SCD and β-Thal. Interestingly, butyrate exposure increased α-globin expression in β-Thal, while α-globin mRNA levels decreased in SCD in response to butyrate. As a result, the favorable effects of the butyrate-induced increase in γ-globin expression on α:β-like globin mRNA imbalance in β-Thal were reduced as a result of the associated increase in α-globin expression. Hemin had similar but less profound effects on all three globin genes in both categories of patients. Although the majority of patients with β-Thal did not correct their globin imbalance in response to butyrate or hemin induction of HbF in a minority of patients resulted in marked reduction in globin imbalance. Thus, we believe that the poor clinical response in a majority of patients with β-Thal to inducers of γ-globin expression may be a reflection of unfavorable effects of these agents on the other globin genes. © 2009 Elsevier Inc. All rights reserved.
dc.format.extent
dc.format.extent Pages: (58-62)
dc.language English
dc.publisher SAN DIEGO
dc.relation.ispartof Publication Name: Blood Cells, Molecules, and Diseases; Publication Year: 2009; Volume: 43; no. 1; Pages: (58-62);
dc.relation.ispartofseries
dc.relation.uri
dc.source Scopus
dc.subject.other
dc.title Differences in response to fetal hemoglobin induction therapy in β-thalassemia and sickle cell disease
dc.type Article
dc.contributor.affiliation Fathallah, H., Mount Sinai School of Medicine, Division of Hematology-Medical Oncology, Box 1079 One, Gustave L. Levy Place, New York, NY 10029, United States
dc.contributor.affiliation Taher, A., Department of Internal Medicine, American University of Beirut, Beirut, Lebanon
dc.contributor.affiliation Bazarbachi, A., Department of Internal Medicine, American University of Beirut, Beirut, Lebanon
dc.contributor.affiliation Atweh, G.F., Mount Sinai School of Medicine, Division of Hematology-Medical Oncology, Box 1079 One, Gustave L. Levy Place, New York, NY 10029, United States
dc.contributor.authorAddress Fathallah, H.; Mount Sinai School of Medicine, Division of Hematology-Medical Oncology, Box 1079 One, Gustave L. Levy Place, New York, NY 10029, United States; email: hassana.fathallah@mssm.edu
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Internal Medicine;
dc.contributor.authorDepartment Internal Medicine
dc.contributor.authorDivision
dc.contributor.authorEmail hassana.fathallah@mssm.edu
dc.contributor.authorFaculty Faculty of Medicine
dc.contributor.authorInitials Fathallah, H
dc.contributor.authorInitials Taher, A
dc.contributor.authorInitials Bazarbachi, A
dc.contributor.authorInitials Atweh, GF
dc.contributor.authorOrcidID
dc.contributor.authorReprintAddress Fathallah, H (reprint author), Mt Sinai Sch Med, Div Hematol Med Oncol, Box 1079,1 Gustave L Levy Pl, New York, NY 10029 USA.
dc.contributor.authorResearcherID Fathallah, Habib-F-3429-2010
dc.contributor.authorUniversity American University of Beirut Medical Center
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dc.description.citedCount 2
dc.description.citedTotWOSCount 4
dc.description.citedWOSCount 3
dc.format.extentCount 5
dc.identifier.articleNo
dc.identifier.coden BCMDF
dc.identifier.pubmedID 19346141
dc.identifier.scopusID 68649098489
dc.identifier.url
dc.publisher.address 525 B ST, STE 1900, SAN DIEGO, CA 92101-4495 USA
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dc.relation.ispartofConferenceDate
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dc.relation.ispartofConferenceTitle
dc.relation.ispartofFundingAgency
dc.relation.ispartOfISOAbbr Blood Cells Mol. Dis.
dc.relation.ispartOfIssue 1
dc.relation.ispartOfPart
dc.relation.ispartofPubTitle Blood Cells, Molecules, and Diseases
dc.relation.ispartofPubTitleAbbr Blood Cells Mol. Dis.
dc.relation.ispartOfSpecialIssue
dc.relation.ispartOfSuppl
dc.relation.ispartOfVolume 43
dc.source.ID WOS:000271076500011
dc.type.publication Journal
dc.subject.otherAuthKeyword Butyrate
dc.subject.otherAuthKeyword Hemin
dc.subject.otherAuthKeyword Hemoglobin expression
dc.subject.otherAuthKeyword Sickle cells
dc.subject.otherAuthKeyword Thalassemia
dc.subject.otherChemCAS butyric acid, 107-92-6, 156-54-7, 461-55-2
dc.subject.otherChemCAS hemin, 16009-13-5
dc.subject.otherChemCAS hemoglobin F, 9034-63-3
dc.subject.otherChemCAS Butyrates
dc.subject.otherChemCAS Fetal Hemoglobin, 9034-63-3
dc.subject.otherChemCAS Hemin, 16009-13-5
dc.subject.otherChemCAS Hemoglobin Subunits
dc.subject.otherChemCAS Hemoglobins
dc.subject.otherChemCAS RNA, Messenger
dc.subject.otherIndex alpha globin
dc.subject.otherIndex beta globin
dc.subject.otherIndex butyric acid
dc.subject.otherIndex gamma globin
dc.subject.otherIndex hemin
dc.subject.otherIndex hemoglobin F
dc.subject.otherIndex adult
dc.subject.otherIndex article
dc.subject.otherIndex beta thalassemia
dc.subject.otherIndex concentration response
dc.subject.otherIndex controlled study
dc.subject.otherIndex drug efficacy
dc.subject.otherIndex erythroid cell
dc.subject.otherIndex female
dc.subject.otherIndex gene expression
dc.subject.otherIndex human
dc.subject.otherIndex human cell
dc.subject.otherIndex male
dc.subject.otherIndex pharmacogenetics
dc.subject.otherIndex priority journal
dc.subject.otherIndex sickle cell anemia
dc.subject.otherIndex Adult
dc.subject.otherIndex Anemia, Sickle Cell
dc.subject.otherIndex beta-Thalassemia
dc.subject.otherIndex Butyrates
dc.subject.otherIndex Cells, Cultured
dc.subject.otherIndex Erythroid Cells
dc.subject.otherIndex Female
dc.subject.otherIndex Fetal Hemoglobin
dc.subject.otherIndex Gene Expression
dc.subject.otherIndex Hemin
dc.subject.otherIndex Hemoglobin Subunits
dc.subject.otherIndex Hemoglobins
dc.subject.otherIndex Humans
dc.subject.otherIndex Male
dc.subject.otherIndex Middle Aged
dc.subject.otherIndex RNA, Messenger
dc.subject.otherIndex Young Adult
dc.subject.otherKeywordPlus GAMMA-GLOBIN SYNTHESIS
dc.subject.otherKeywordPlus PHARMACOLOGICAL INDUCTION
dc.subject.otherKeywordPlus ERYTHROID PRECURSORS
dc.subject.otherKeywordPlus HYDROXYUREA THERAPY
dc.subject.otherKeywordPlus INCREASES
dc.subject.otherKeywordPlus ANEMIA
dc.subject.otherKeywordPlus INTERMEDIA
dc.subject.otherKeywordPlus INDUCERS
dc.subject.otherWOS Hematology


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