Two novel arrangements of the human fetal globin genes: Gγ-Gγand Aγ-Aγ
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چکیده
منابع مشابه
Persistent Fetal γ-Globin Expression in Adult Transgenic Mice following Deletion of Two Silencer Elements Located 3′ to the Human Aγ-Globin Gene
Human β-globin gene expression is regulated tightly during development and hematopoiesis. The human β-globin locus comprises five developmentally regulated genes (5′-ε-Gγ-Aγ-δ-β-3′) whose high level and stage-specific expression depends on interactions with the locus control region (LCR), consisting of five major DNaseI hypersensitive sites (Figure 1). The LCR activates β-globin gene transcript...
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Reactivation of fetal hemoglobin genes has been proposed as a potential therapeutic procedure in patients with β-thalassemia, sickle cell disease or other βhemoglobinopathies. In vitro model systems based on small plasmid globin gene constructs have previously been used in human and mouse erythroleukemic cell lines to study the molecular mechanisms regulating the expression of the fetal human g...
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Background: In the previous study, we have shown that the presence of A allele at position -588 in Aγ-globin gene was highly frequent and closely associated with fetal hemoglobin elevation among β-thalassemia intermedia patients. Therefore, we decided to investigate whether this allele (A allele at -588) could result in an increase in Aγ-globin gene expression to ameliorate the severity of the ...
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Natural deletions of the human β-globin gene cluster lead to specific syndromes characterized by increased production of fetal hemoglobin in adult life and provide a useful model to delineate novel cis-acting elements involved in the developmental control of hemoglobin switching. A hypothesis accounting for these phenotypic features, assumes that silencers located within the Aγ to δ-gene region...
متن کاملIntervening Sequences in Human Fetal Globin Genes
The large intervening sequences (IVSB) of three human fetal globin genes contain tracts of alternating purine-pyrimidine sequences approximately 40-60 base pairs in length which adopt left-handed Z DNA helices under the influence of negative supercoiling. The amount of negative supercoiling (-0.045) required for the rightto left-handed transitions is within the physiological range. The structur...
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ژورنال
عنوان ژورنال: Nucleic Acids Research
سال: 1984
ISSN: 0305-1048,1362-4962
DOI: 10.1093/nar/12.18.7023