Diseases impacting the production, longevity, and function of the hemoglobin molecule are
diverse in their impact on an individual and population basis. This article focuses on sickle …

Red blood cells (RBC) are specifically differentiated to transport oxygen and carbon dioxide
in the blood and they lack most organelles, including mitochondria. The autoxidation of …

Hydroxyurea (HU) is the sole approved pharmacological therapy for sickle cell disease
(SCD). Higher levels of fetal hemoglobin (HbF) diminish deoxygenated sickle globin …

Abstract β-thalassemia is caused by mutations in the β-globin locus resulting in loss of, or
reduced, hemoglobin A (adult hemoglobin, HbA, α2β2) production. Hydroxyurea treatment …

Therapeutic regulation of globin genes is a primary goal of translational research aimed
toward hemoglobinopathies. Signal transduction was used to identify chromatin …

The molecular mechanisms governing γ-globin expression in a subset of fetal hemoglobin
(α2γ2: HbF) expressing red blood cells (F-cells) and the mechanisms underlying the …

Background Sickle cell disease (SCD) is a fatal monogenic disorder with no effective cure
and thus high rates of morbidity and sequelae. Efforts toward discovery of disease modifying …

The natural history of severe hemoglobinopathies like sickle cell disease (SCD) is rather
variable, depending on the circumstances, but the main influence on such variability is the …

The major effect associated with hydroxyurea (HU) treatment of sickle cell anaemia (SCA)
patients is an increase in fetal haemoglobin (HbF) synthesis, which inhibits the …

The concentration of circulating hematopoietic stem and progenitor cells has not been
studied longitudinally. Here, we report that the proportions of Lin-CD34+ 38-hematopoietic …