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Breaking Free from Your Fetal Chains: A Case-Based Review of the Literature on Gamma Chain Variant Hemoglobinopathies [PDF]

open access: yesNeonatal Medicine, 2023
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

HRI-regulated transcription factor ATF4 activates BCL11A transcription to silence fetal hemoglobin expression.

open access: yesBlood, 2020
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

Induction of fetal hemoglobin: Lentiviral shRNA knockdown of HBS1L in β0-thalassemia/HbE erythroid cells.

open access: yesPLoS ONE, 2023
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]

open access: yes, 2016
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

Comparative targeting analysis of KLF1, BCL11A, and HBG1/2 in CD34+ HSPCs by CRISPR/Cas9 for the induction of fetal hemoglobin

open access: yesScientific Reports, 2020
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

open access: yes陆军军医大学学报, 2023
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]

open access: yes, 2017
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

open access: yesHaematologica, 2020
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

open access: yesBrazilian Journal of Medical and Biological Research, 2003
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

open access: yesHaematologica, 2020
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

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