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|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.|
Finzi Corat M.A.
Barbosa De Melo M.
Corra Passos L.A.
|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.|
|Appears in Collections:||Unicamp - Artigos e Outros Documentos|
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