From a normal human brain phage display library screen we identified the gamma (A)-globin chain of fetal hemoglobin (Hb F) as a protein that bound strongly to A beta1-42. We showed the oxidized form of adult Hb (metHb A) binds with greater affinity to A beta1-42 than metHb F. MetHb is more toxic than oxyhemoglobin because it loses its heme group more readily. Free Hb and heme readily damage vas...

High levels of fetal hemoglobin (Hb F) protect from many of the complications of sickle cell disease and lead to improved survival. Butyrate and other short chain fatty acids were previously shown to increase Hb F production in erythroid cells in vitro and in animal models in vivo. However, butyrates are also known to inhibit the proliferation of many cell types, including erythroid cells. Expe...

Heme toxicity contributes to the pathogenesis of chronic inflammatory diseases, atherosclerosis, and hemolysis associated vasculopathy. Macrophage clearance of cell free hemoglobin (Hb) is thus an essential homeostatic function of these cells. We examined the transcriptional response of human PBMC derived macrophages to Hb by gene array analysis. The observed noninflammatory macrophage response...

Fetal hemoglobin (Hb F) is an important genetic modulator of the beta-hemoglobinopathies. The regulation of Hb F levels is influenced by transcription factors. We used phylogenetic footprinting to screen transcription factors that have binding sites in HBG1 and HBG2 genes' noncoding regions in order to know the genetic determinants of the Hb F expression. Our analysis showed 354 conserved motif...

Hb F, some of which is glycated (2), accounts for the majority (70%–95%) of Hb species in newborns. About 5%– 30% is Hb A, with none to trace amounts of Hb A1c. This patient’s sample was collected after transfusion of 2 aliquots of red blood cells from a single donor. The donor’s blood contained 12.6% Hb A1c. Concentrated glucose in blood storage media increases donor Hb A1c only minimally (3, ...

Give a brief description of Haemoglobin (Hb):  Hb is made up of Four Subunits (Tetramer) held together by multiple non-covalent interactions  Each subunit (Monomer) consist of: Haem (Ferro-Protoporphyrin) and Globin protein  Haem consist of a Protoporphyrin IX and Ferrous ion (Fe 2 )  Globin protein folds around the Haem group forming a hydrophobic pocket that protects the Haem, which serve...

We report a case of beta-thalassemia/Hb Tak compound heterozygote. The 7 year-old Thai boy presented with plethora since birth. Hemoglobin electrophoresis showed a major band between Hb A2 and Hb F and absent Hb A. DNA sequencing study demonstrated an AC insertion at the terminal codon of the beta-globin gene. The clinical feature of polycythemia reflected a high oxygen affinity of Hb Tak.

In hereditary persistence of fetal hemoglobin, Hb F (alpha 2 gamma 2) is elevated after birth. Screening of sickle cell patients has revealed a family with elevated Hb F and high A gamma values. The propositus was a sickle cell patient with approximately 25% Hb F and 68.4% A gamma. He was heterozygous for the Benin (#19) and Mor beta S haplotypes. Five AS relatives with the Mor haplotype had 2....

conclusions: hydroxyurea can be safely used in some transfusion-dependent β-thalassemia patients to decrease their transfusion needs. background: β-thalassemia is an inherited hemoglobin disorder caused by defective synthesis of ß-globin chains. hemoglobin (hb) f induction is a possible therapeutic approach which can partially compensate for α and non-α globin chains imbalance. objectives: we a...