Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/92600
Type: Artigo de periódico
Title: High Levels Of Human γ-globin Are Expressed In Adult Mice Carrying A Transgene Of The Brazilian Type Of Hereditary Persistence Of Fetal Hemoglobin (aγ -195)
Author: Da Cunha A.F.
Brugnerotto A.F.
Finzi Corat M.A.
Devlin E.E.
Gimenes A.P.
Barbosa De Melo M.
Corra Passos L.A.
Bodine D.
Saad S.T.O.
Costa F.F.
Abstract: Hereditary persistence of fetal hemoglobin (HPFH) is characterized by increased levels of Hb F during adult life. Nondeletional forms of HPFH are characterized by single base mutations in the Aγ and Gγ promoters, resulting in an increase of Hb F ranging from 3 to 20 in heterozygotes. Many point mutations in this region have been described, including the Aγ -195 (C>G) mutation that causes the Brazilian type of HPFH (HPFH-B). To better understand this mechanism, we have developed HPFH-B transgenic mice. mRNA levels of human γ-globin of -195 transgenic mice were clearly higher when compared with control transgenic mice bearing a wild type sequence of the γ promoter. Thus, our data indicate that the -195 mutation is the unique cause of elevation of Hb F in Brazilian HPFH. These results could provide us with an opportunity to study the modifying effects of the Hb F in the phenotype of sickle cell disease and β-thalassemia (β-thal). © Informa UK Ltd.
Editor: 
Rights: fechado
Identifier DOI: 10.3109/03630260903344176
Address: http://www.scopus.com/inward/record.url?eid=2-s2.0-72049086207&partnerID=40&md5=2a3401e0eebca36433bb97da45e71ef9
Date Issue: 2009
Appears in Collections:Unicamp - Artigos e Outros Documentos

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