Publisher Summary The chapter describes the understanding of the physics and physical
chemistry of sickle cell hemoglobin polymerization in solutions and in red cells. The …

Nonspecific interactions between individual macro-molecules and their immediate
surroundings (background interactions') within a medium as heterogeneous and highly …

Patients with homozygous sickle-cell disease may be homozygous for alpha-thalassemia 2
(α–/α–), may be heterozygous for alpha-thalassemia 2 (α–/αα), or may have a normal alpha …

The thermodynamics of sickle cell hemoglobin gelation in the presence of oxygen has been
investigated by measuring the fractional saturation of the solution and polymer phases, and …

Sickle cell anemia (SCA) is an inherited disorder of hemoglobin synthesis that is
characterized by life‐long severe hemolytic anemia, attacks of pain crisis, and chronic organ …

Abstract We have established that 2, 3-diphosphoglycerate (2, 3-DPG) content and
intracellular pH exert separate, but interdependent, effects on the equilibrium solubility (csat) …

Because the formation of sickle cells is dependent on the intracellular concentration of
deoxyhemoglobin S, we investigated the possibility of altering or preventing sickle-cell …

The primary pathophysiological event in the erythrocytes of individuals with the various
sickle syndromes is the intracellular aggregation or polymerization of sickle haemoglobin …

Erythropoietin is being used more widely in the management of sickle cell disease (SCD,
inclusive of homozygous sickle beta, SS, and compound heterozygous sickle beta …

Although highly charged polyanions, such as inositol hexaphosphate, have been clearly
shown to decrease the solubility of deoxyhemoglobin S, the effect of 2, 3 …