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).

dc.creatorda Cunha, Anderson F
dc.creatorBrugnerotto, Ana F
dc.creatorCorat, Marcus A Finzi
dc.creatorDevlin, Emily E
dc.creatorGimenes, Ana P
dc.creatorde Melo, Mônica Barbosa
dc.creatorPassos, Luiz A Corrêa
dc.creatorBodine, David
dc.creatorSaad, Sara T O
dc.creatorCosta, Fernando F
dc.date2009
dc.date2015-11-27T13:15:43Z
dc.date2015-11-27T13:15:43Z
dc.date.accessioned2018-03-29T01:09:43Z
dc.date.available2018-03-29T01:09:43Z
dc.identifierHemoglobin. v. 33, n. 6, p. 439-47, 2009.
dc.identifier1532-432X
dc.identifier10.3109/03630260903344176
dc.identifierhttp://www.ncbi.nlm.nih.gov/pubmed/19958189
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/198547
dc.identifier19958189
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1298780
dc.descriptionHereditary 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).
dc.description33
dc.description439-47
dc.languageeng
dc.relationHemoglobin
dc.relationHemoglobin
dc.rightsfechado
dc.rights
dc.sourcePubMed
dc.subjectAnemia, Sickle Cell
dc.subjectAnimals
dc.subjectBrazil
dc.subjectFetal Hemoglobin
dc.subjectHumans
dc.subjectMice
dc.subjectMice, Transgenic
dc.subjectMutation
dc.subjectPhenotype
dc.subjectRna, Messenger
dc.subjectTransgenes
dc.subjectBeta-thalassemia
dc.subjectGamma-globins
dc.titleHigh 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).
dc.typeArtículos de revistas


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