Disruption of the BCL11A Erythroid Enhancer Reactivates Fetal Hemoglobin in Erythroid Cells of Patients with β-Thalassemia Major
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چکیده
منابع مشابه
Fetal hemoglobin synthesis in erythroid cultures in hereditary persistence of fetal hemoglobin and beta o-thalassemia.
To determine whether hemoglobin regulation is normal in diseases affecting beta-globin gene expression, globin synthesis was examined in members of a family of a patient with hereditary persistence of fetal hemoglobin/beta o-thalassemia (HPFH/beta o-thal). The HPFH defect is the Ghanian type II, with a deletion from psi beta 1 to at least 20 kb 3' to beta. The beta o-thal gene has the haplotype...
متن کاملResveratrol: Antioxidant activity and induction of fetal hemoglobin in erythroid cells from normal donors and β-thalassemia patients.
Thalassemia and sickle-cell anemia (SCA) present a major public health problem in countries where the number of carriers and affected individuals is high. As a result of the abnormalities in hemoglobin production, cells of thalassemia and SCA patients exhibit oxidative stress, which ultimately is responsible for the chronic anemia observed. Theref...
متن کاملFetal Hemoglobin Synthesis in Erythroid Cultures in Hereditary Persistence of Fetal Hemoglobin and @#{176}-Thalassemia
To determine whether hemoglobin regulation is normal in diseases affecting 1-globin gene expression. globin synthesis was examined in members of a family of a patient with hereditary persistence of fetal hemoglobin/fi#{176}-thalassemia (HPFH/$#{176}-thal). The HPFH defect is the Ghanian type II. with a deletion from t’$ to at least 20 kb 3’ to . The / #{176}-thaI gene has the haplotype II restr...
متن کاملStochastic expression of fetal hemoglobin in adult erythroid cells.
The expression of fetal hemoglobin, Hb F, in the adult cells is cellularly restricted both in vivo and in culture. Because, in cultures of erythroid progenitors, subclones that express or fail to express Hb F are derived from the same erythroid stem cell, a mechanism must exist whereby Hb F expression segregates in the progeny of erythroid progenitors during their differentiation. We present ma...
متن کاملA common signaling pathway is activated in erythroid cells expressing high levels of fetal hemoglobin: a potential role for cAMP-elevating agents in β-globin disorders
BACKGROUND Although erythroid cells prepared from fetal liver, cord blood, or blood from β-thalassemia patients are known to express fetal hemoglobin at high levels, the underlying mechanisms remain elusive. We previously showed that cyclic nucleotides such as cAMP and cGMP induce fetal hemoglobin expression in primary erythroid cells. Here we report that cAMP signaling contributes to high-leve...
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ژورنال
عنوان ژورنال: Molecular Therapy - Methods & Clinical Development
سال: 2018
ISSN: 2329-0501
DOI: 10.1016/j.omtm.2018.08.003