نتایج جستجو برای: fetal hemoglobin hbf
تعداد نتایج: 142698 فیلتر نتایج به سال:
To the Editor: Regulation of fetal hemoglobin (HbF) production is complex [1]. Our experience of persistent HbF elevation during a 7-year follow up in a child with homozygous sickle cell disease (SCD) who underwent chemotherapy for Wilms tumor (WT) adds to the complexity of the regulatory mechanisms in HbF production. An 8-year-old African–American female homozygous SCD patient with metastatic ...
Thalassemia, an inherited quantitative globin disorder, consists of two types, α– and β–thalassemia. β–thalassemia is a heterogeneous disease that can be asymptomatic, mild, or even severe. Considerable research has focused on investigating its underlying etiology. These studies found DNA hypomethylation in the β–globin gene cluster significantly related to fetal hemoglobin (HbF) elevation. His...
The design and evaluation of therapies for the sickle cell and beta-thalassemia syndromes rely on our understanding of hemoglobin accumulation during human erythropoiesis. Here we report direct measurements of hemoglobin composition and messenger RNA (mRNA) levels in cultured CD34(+) cells and correlate those measurements with studies of freshly obtained bone marrow samples. Hemoglobin levels i...
Background: β-thalassemia is the most common monogenic disorder in human. The (CT) polymorphism at -158 upstream region of the γ-globin gene and pharmacological factors such as hydroxyurea have been reported to influence γ-globin gene expression and the severity of clinical symptoms of β-thalassemia. Methods: In the present study, 51 βthalassemia intermediate patients were studied. Xmn1γ polym...
Fetal hemoglobin (HbF) is regulated as a multigenic trait. By genome-wide association study, we confirmed that HBS1L-MYB intergenic polymorphisms (HMIP) and BCL11A polymorphisms are highly associated with HbF in Chinese β-thalassemia heterozygotes. In this population, the variance in HbF resulting from the HMIP is 13.5%; that resulting from the BCL11A polymorphism is 6.4%. To identify the funct...
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...
Genetic polymorphisms in Quantitative Trait Loci (QTL) genes such as BCL11A, HBS1L-MYB and KLF1 have been reported to influence fetal hemoglobin (HbF) levels. This prospective study was planned evaluate the role of genetic QTL determinant HbF levels beta thalassemia major patients. The carried out on 100 Blood samples were collected EDTA plain vials for biochemical molecular evaluation. genotyp...
Genome editing (GE) is one of the most efficient and useful molecular approaches to correct effects gene mutations in hereditary monogenetic diseases, including β-thalassemia. CRISPR-Cas9 has been proposed for effective correction β-thalassemia mutation, obtaining high-level “ de novo ” production adult hemoglobin (HbA). In addition primary causing β-thalassemia, several reports demonstrate tha...
Single-cell microscopic immunodiffusion assays were used to determine the cellular mechanisms that regulate fetal hemoglobin (HbF) levels in cultures of primitive and late erythroid precursors obtained from human adult bone marrow. Two variables--the percentage of cells containing HbF (F cells) and the picograms (pg) of HbF/F cell--were assayed in cells derived from erythroid colony-forming uni...
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