Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/198547
Type: Artigo de periódico
Title: High Levels Of Human Gamma-globin Are Expressed In Adult Mice Carrying A Transgene Of The Brazilian Type Of Hereditary Persistence Of Fetal Hemoglobin ((a)gamma -195).
Author: da Cunha, Anderson F
Brugnerotto, Ana F
Corat, Marcus A Finzi
Devlin, Emily E
Gimenes, Ana P
de Melo, Mônica Barbosa
Passos, Luiz A Corrêa
Bodine, David
Saad, Sara T O
Costa, Fernando 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 (A)gamma and (G)gamma 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 (A)gamma -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 gamma-globin of -195 transgenic mice were clearly higher when compared with control transgenic mice bearing a wild type sequence of the gamma 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 beta-thalassemia (beta-thal).
Subject: Anemia, Sickle Cell
Animals
Brazil
Fetal Hemoglobin
Humans
Mice
Mice, Transgenic
Mutation
Phenotype
Rna, Messenger
Transgenes
Beta-thalassemia
Gamma-globins
Rights: fechado
Identifier DOI: 10.3109/03630260903344176
Address: http://www.ncbi.nlm.nih.gov/pubmed/19958189
Date Issue: 2009
Appears in Collections:Artigos e Materiais de Revistas Científicas - Unicamp

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