Induction of fetal hemoglobin (Hb F) is an important therapeutic tool in ameliorating complications of sickle cell disease. Nitric oxide has been implicated in the mechanism of Hb F synthesis induced by hydroxyurea (HU). This study examined whether zileuton (ZL), a structural analog of hydroxyurea, possessed Hb F-inducing properties and the potential role nitric oxide plays. ZL caused a dose-de...

objective(s): β-thalassemia is one of the most common genetic disorders in the world. as one of the promising treatment strategies, fetal hemoglobin (hb f) can be induced. the present study was an attempt to reactivate the γ-globin gene by introducing a gene construct containing klf1 binding sites to the k562 cell line. materials and methods: a plasmid containing a 192 bp sequence with two repe...

THIS ISSUE OF BLOOD features a report by Atweh et al1 on the induction of fetal hemoglobin (Hb F) by pulse butyrate. This study is a solid addition to the recent and gratifying momentum in the development of effective therapy for sickle cell disease. Underlying this report is a series of novel and convincing in vivo studies extending from model systems in the chicken,2 sheep,3 and baboons4-6 to...

background: there are many parameters that modulate the severity of sickle cell anemia. fetal hemoglobin (hb f) is one of these major variables. however, its effect is clinically inconsistent. we conducted a descriptive study to assess the influence of hb f on clinical events and hematological variables in patients with sickle cell anemia. methods: 151 patients with sickle cell anemia with a st...

To clarify the role of gammaN-terminal Gly, gamma5 Glu, and gamma143 Ser in 2,3-biphosphosphoglycerate (BPG) binding to fetal hemoglobin (Hb F), we engineered and produced normal human Hb F and two Hb F variants (Hb F gammaG1V, gammaS143H, and Hb F gammaG1V, gammaE5P, gammaS143H) using a yeast expression system and then compared their oxygen-binding properties with those of native human Hb F an...

To further understand determinants for Hemoglobin (Hb) S polymerization, as well as the inhibitory mechanism of Hb F on Hb S polymerization, Hb F variants containing Val-* (Hb F yE6V) or Val-*, Thr-y87 (Hb F yE6V. yQ87Tj were expressed in yeast. The oxy form of Hb F yE6V was about 10-fold less stable to mechanical agitation than native oxy Hb F, which is similar to stability differences compari...

Background: There are many parameters that modulate the severity of sickle cell anemia. Fetal hemoglobin (Hb F) is one of these major variables. However, its effect is clinically inconsistent. We conducted a descriptive study to assess the influence of Hb F on clinical events and hematological variables in patients with sickle cell anemia. Methods: 151 patients with sickle cell anemia with a st...

Neonatal cyanosis in healthy newborns can be associated either with methemoglobin due to cytochrome b5 reductase deficiency or to M-hemoglobin, a group of hemoglobin variants resulting from mutations in the globin chain genes. We report the clinical case of a neonate with cyanosis and normal cardiac and respiratory function. At birth the hematological parameters were normal; however, the methem...

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...