Breaking Free from Your Fetal Chains: A Case-Based Review of the Literature on Gamma Chain Variant Hemoglobinopathies [PDF]
Methemoglobinemia is a dyshemoglobinemia characterized by cyanosis and reduced oxygen saturation with increased methemoglobin values. The etiology may be congenital or acquired, with the latter being more common.
Pearl Mary Varughese+3 more
doaj +1 more source
Reactivation of fetal hemoglobin remains a critical goal in the treatment of patients with sickle cell disease and β-thalassemia. Previously, we discovered that silencing of the fetal γ-globin gene requires the erythroid-specific eIF2α kinase HRI ...
Peng Huang+12 more
semanticscholar +1 more source
Imbalanced globin chain output contributes to thalassemia pathophysiology. Hence, induction of fetal hemoglobin in β-thalassemia and other β-hemoglobinopathies are of continuing interest for therapeutic approaches.
Sukanya Chumchuen+4 more
doaj +1 more source
MR imaging–derived oxygen-hemoglobin dissociation curves and fetal-placental oxygen-hemoglobin affinities [PDF]
PURPOSE: To generate magnetic resonance (MR) imaging–derived, oxygen-hemoglobin dissociation curves and to map fetal-placental oxygen-hemoglobin affinity in pregnant mice noninvasively by combining blood oxygen level–dependent (BOLD) T2* and oxygen ...
Akselrod-Ballin, Ayelet+6 more
core +2 more sources
β-hemoglobinopathies are caused by abnormal or absent production of hemoglobin in the blood due to mutations in the β-globin gene (HBB). Imbalanced expression of adult hemoglobin (HbA) induces strong anemia in patients suffering from the disease. However,
Andrés Lamsfus-Calle+12 more
semanticscholar +1 more source
Hydroxyurea ameliorates mouse hypoxia through inducing embryonic hemoglobin
Objective To investigate the ameliorative effect and mechanism of hydroxyurea (HU) on hypoxia mice in a closed environment. Methods Sixty male C57BL/6J mice (6~8 weeks old, weighing 18~22 g) were randomly divided into normoxia control group (NC, n=10 ...
ZHOU Xiaoying+4 more
doaj +1 more source
Whole transcriptome analysis of human erythropoietic cells during ontogenesis suggests a role of VEGFA gene as modulator of fetal hemoglobin and pharmacogenomic biomarker of treatment response to hydroxyurea in β-type hemoglobinopathy patients [PDF]
Background: Human erythropoiesis is characterized by distinct gene expression profiles at various developmental stages. Previous studies suggest that fetal-to-adult hemoglobin switch is regulated by a complex mechanism, in which many key players still ...
Ali, Bassam R.+11 more
core +4 more sources
In vitro and in vivo induction of fetal hemoglobin with a reversible and selective DNMT1 inhibitor
Pharmacological induction of fetal hemoglobin (HbF) expression is an effective therapeutic strategy for the management of β-hemoglobinopathies such as sickle cell disease.
A. Gilmartin+22 more
semanticscholar +1 more source
Effect of hydroxyurea on G gamma chain fetal hemoglobin synthesis by sickle-cell disease patients
Hydroxyurea is used for sickle-cell disease patients in order to increase fetal hemoglobin synthesis and consequently decrease the severity of pain episodes.
S.M. Teixeira+2 more
doaj +1 more source
Fetal hemoglobin rescues ineffective erythropoiesis in sickle cell disease
While ineffective erythropoiesis has long been recognized as a key contributor to anemia in thalassemia, its role in anemia of sickle cell disease (SCD) has not been critically explored.
Sara El Hoss+13 more
semanticscholar +1 more source