Solubilities of deoxygenated sickle cell hemoglobin (deoxy-Hb S), at varying pH and temperature over a range of concentrations encompassing those found in erythrocytes, were measured. The technique involved ultracentrifugation, which gave values of the supernatant concentration and the mass of the sedimented material. The data establish that the solubility of doexy-Hb S is the saturation concentration and is independent of initial concentration. The mass of the pellet phase increases linearly with initial concentration. Moreover, the saturation concentration represents the critical concentration above which monomers are in equilibrium with polymers. These polymers are the putative cause of erythrocytes deformation associated with sickle cell anemia. The solubility-pH profiles of deoxy-Hb S at various temperatures, unlike those of other proteins, show no minima at the isoelectric pH but instead show a marked decrease in solubility below pH 7.0, indicating the predominance of polymerization over the expected increase in solubility. Deoxy-Hb S, within specified ranges of temperature and pH, possesses a negative temperature coefficient of solubility, a property characteristic of hydrophobic interactions. The saturation concentratio...Continue Reading
Structure of hemoglobin S fibers: optical determination of the molecular orientation in sickled erythrocytes
Microtubules from mammalian brain: some properties of their depolymerization products and a proposed mechanism of assembly and disassembly
Kinetics and mechanism of deoxyhemoglobin S gelation: a new approach to understanding sickle cell disease
Inhibition of hemoglobin S polymerization by N-terminal band 3 peptides: new class of inhibitors: solubility studies
Modification of hemoglobin S with dimethyl adipimidate. Contribution of individual reacted subunits to changes in properties
Dependence on pH of formation and oxygen affinity of hemoglobin S fibers in the presence and absence of phosphates and polyphosphates
Self-assembled multi-component catenanes: the effect of multivalency and cooperativity on structure and stability
Disagreement between calorimetric and van't Hoff enthalpies of assembly of protein supramolecular structures
Sparing effect of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S at physiologic ligand saturations
Development of antisickling compounds that chemically modify hemoglobin S specifically within the 2,3-diphosphoglycerate binding site
Gelation of sickle cell hemoglobin. IV. Phase transitions in hemoglobin S gels: separate measures of aggregation and solution--gel equilibrium
Noncovalent inhibitors of sickle hemoglobin gelation: effects of aliphatic alcohols, amides, and ureas
Quantitative assessment of the noncovalent inhibition of sickle hemoglobin gelation by phenyl derivatives and other known agents
Mechanism for the increase in solubility of deoxyhemoglobin S due to cross-linking the beta chains between lysine-82 beta 1 and lysine-82 beta 2
Moderate and intense muscular exercises induce marked intramyocellular metabolic acidosis in sickle cell disease mice
Polymer structure and solubility of deoxyhemoglobin S in the presence of high concentrations of volume-excluding 70-kDa dextran. Effects of non-s hemoglobins and inhibitors.
Blood And Marrow Transplantation
The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.
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