Hemoglobin S gelation and sickle cell disease

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5-Azacytidine increases gamma-globin synthesis and reduces the proportion of dense cells in patients with sickle cell anemia

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Erythrocytes in sickle cell anemia are heterogeneous in their rheological and hemodynamic characteristics.

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Delay Time of Hemoglobin S Polymerization Prevents Most Cells from Sickling in Vivo

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The intracellular polymerization of sickle hemoglobin and its relevance to sickle cell disease

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Supersaturation in sickle cell hemoglobin solutions.

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Hemoglobin S polymerization: primary determinant of the hemolytic and clinical severity of the sickling syndromes

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Structural bases of the inhibitory effects of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S

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[16] Polarized absorption and linear dichroism spectroscopy of hemoglobin

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Erythrocyte Hb-S Concentration AN IMPORTANT FACTOR IN THE LOW OXYGEN AFFINITY OF BLOOD IN SICKLE CELL ANEMIA

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Kinetics of nucleation-controlled polymerization. A perturbation treatment for use with a secondary pathway

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Editorial: Delay time of gelation: a possible determinant of clinical severity in sickle cell disease

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Three-dimensional reconstruction of the fibres of sickle cell haemoglobin

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Effects of thalassemia and microcytosis on the hematologic and vasoocclusive severity of sickle cell anemia

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Thermodynamics of gelation of sickle cell deoxyhemoglobin

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Adherence of sickle erythrocytes to vascular endothelial cells: requirement for both cell membrane changes and plasma factors

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Ligand-induced conformational dependence of hemoglobin in sickling interactions

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Periodic Microcirculatory Flow in Patients with Sickle-Cell Disease

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Requirements for therapeutic inhibition of sickle haemoglobin gelation

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Properties of sickle-cell haemoglobin

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Hemoglobin Interaction: Modification of Solid Phase Composition in the Sickling Phenomenon

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Nucleation-controlled aggregation of deoxyhemoglobin S. Possible difference in the size of nuclei in different phosphate concentrations.

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Hydroxyurea induction of hemoglobin F production in sickle cell disease: relationship between cytotoxicity and F cell production

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Effects of pH, 2,3-diphosphoglycerate and salts on gelation of sickle cell deoxyhemoglobin

Briehl, R. W., & Ewert, S. M. (1973) · Journal of Molecular Biology · doi:10.1016/0022-2836(73)90415-4 · Cited by 122

A Study of Induced Hyponatremia in the Prevention and Treatment of Sickle-Cell Crisis

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Oxygen binding by sickle cell hemoglobin polymers

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Asymmetrical hemoglobin hybrids. Approach to the study of subunit interactions

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Subunit assembly of hemoglobin: an important determinant of hematologic phenotype

Bunn, H. F. (1987) · Blood · doi:10.1182/blood.v69.1.1.1 · Cited by 114

Hemolysis in Sickle Cell Disease

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Dense cells in sickle cell anemia: the effects of gene interaction

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LINEAR DICHROISM OF BIOLOGICAL CHROMOPHORES

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Heterogeneity of red cells in the sickler: a characteristic with practical clinical and pathophysiological implications.

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Sickle Hemoglobin Polymerization in Solution and in Cells

Noguchi, C. T., & Schechter, A. N. (1985) · Annual Review of Biophysics and Biophysical Chemistry · doi:10.1146/annurev.bb.14.060185.001323 · Cited by 108

Vaso-occlusion by sickle cells: evidence for selective trapping of dense red cells

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Intrinsic microvascular control of tissue oxygen delivery

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Modulation of erythrocyte-endothelial interactions and the vasocclusive severity of sickling disorders

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Kinetics of sickle haemoglobin polymerization in single red cells

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Irreversibly sickled cells and red cell survival in sickle cell anemia

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β-Chain contact sites in the haemoglobin S polymer

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REFINED CRYSTAL STRUCTURE OF DEOXYHEMOGLOBIN S. I. RESTRAINED LEAST-SQUARES REFINEMENT AT 3.0-ANGSTROMS RESOLUTION

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A temperature-dependent latent-period in the aggregation of sickle-cell deoxyhemoglobin

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Alpha-thalassemia is related to prolonged survival in sickle cell anemia

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Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.

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Hemodynamic Studies in Sickle Cell Anemia

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Thermodynamic studies of polymerization of deoxygenated sickle cell hemoglobin.

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The rates of polymerization and depolymerization of sickle cell hemoglobin

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Cardiac function in sickle cell anemia

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Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

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Calorimetric and optical characterization of sickle cell hemoglobin gelation

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O-Thalassemia Reduces the Hemolytic Rate in Homozygous Sickle-Cell Disease

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A STUDY OF CARDIOPULMONARY ALTERATIONS IN PATIENTS WITH SICKLE CELL DISEASE AND ITS VARIANTS1

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Influence of oxygen tension on the viscoelastic behavior of red blood cells in sickle cell disease

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Physiological and x-ray studies of potential antisickling agents.

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The percentage of dense red cells does not predict incidence of sickle cell painful crisis

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Clinical diversity of sickle cell anemia: Genetic and cellular modulation of disease severity

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Molecular topology in crystals and fibers of hemoglobin S

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Solubilization of hemoglobin S by other hemoglobins.

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Cardiorespiratory adjustments in chronic sickle cell anemia.

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Crystallization of sickle hemoglobin from gently agitated solutions—An alternative to gelation

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Ligand binding and the gelation of sickle cell hemoglobin

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5-Azacytidine increases HbF production and reduces anemia in sickle cell disease: dose-response analysis of subcutaneous and oral dosage regimens

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The kinetics of the sol-gel transformation of deoxyhemoglobin S by continuous monitoring of viscosity.

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Cell heterogeneity in sickle cell disease: quantitation of the erythrocyte density profile.

Rodgers, G. P., Schechter, A. N., & Ct, N. (1985) · PubMed · Cited by 64

Concerted Formation of the Gel of Hemoglobin S

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Electrostatic attraction governs the dimer assembly of human hemoglobin.

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Effects of amino acids on gelation kinetics and solubility of sickle hemoglobin

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Improved exercise performance after exchange transfusion in subjects with sickle cell anemia

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Relationship between morphologic characteristics of sickle cells and method of deoxygenation.

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Oxygen binding to sickle cell hemoglobin

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In vivo study of the sickle cell phenomenon.

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Deoxygenated sickle hemoglobin. Effects of lyotropic salts on its solubility.

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Calculated dispersion of capillary transit times: significance for oxygen exchange

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Formation of needle-like aggregates in stirred solutions of hemoglobin S

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A simple laboratory alternative to irreversibly sickled cell (ISC) counts

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Polymerization of hemoglobin in sickle trait erythrocytes and lysates.

Noguchi, C. T., Torchia, D. A., & Schechter, A. N. (1981) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)69413-5 · Cited by 47

Rational Approaches to Chemotherapy: Antisickling Agents

Klotz, I. M., Haney, D. N., & King, L. C. (1981) · Science · doi:10.1126/science.7256275 · Cited by 47

Kinetics of hemoglobin S gelation followed by continuously sensitive low-shear viscosity

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Sickling times of individual erythrocytes at zero Po2.

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Cardiovascular findings in children with sickle cell anemia

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Alternative aspirins as antisickling agents: acetyl-3,5-dibromosalicylic acid.

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THE ALVEOLAR-ARTERIAL OXYGEN PRESSURE GRADIENT IN ANEMIA 1

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Lung function in children with sickle cell anemia.

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Comparative evaluation of fifteen anti-sickling agents

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KINETICS OF GAS TRANSPORT IN THE BLOOD

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Oxygen Transport in Sickle Cell Anemia

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Gelation of Sickle Cell Hemoglobin: Effects of Hybrid Tetramer Formation in Hemoglobin Mixtures

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Ultrastructure of Sickling and Unsickling in Time‐Lapse Studies

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Pressure effects on the flow behavior of sickle (HbSS) red cells in isolated (ex-vivo) microvascular system

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ARTERIAL BLOOD OXYGENATION IN SICKLE CELL ANEMIA*

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Demonstration of a delay time during aggregation of diluted solutions of deoxyhemoglobin S and hemoglobin CHarlem in concentrated phosphate buffer.

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The effect of cell hydration on the deformability of normal and sickle erythrocytes

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Thermodynamic analysis of the chemical inhibition of Sickle-Cell hemoglobin gelation

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Gelation of sickle cell hemoglobin IV. Phase transitions in hemoglobin S gels: Separate measures of aggregation and solution-gel equilibrium

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Failure of desmopressin to lower serum sodium or prevent crisis in patients with sickle cell anemia

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