Hemoglobin S gelation and sickle cell disease

ON THE NATURE OF ALLOSTERIC TRANSITIONS: A PLAUSIBLE MODEL.

Monod, J., Wyman, J., & Changeux, J. (1965) · Journal of Molecular Biology · doi:10.1016/s0022-2836(65)80285-6 · Cited by 7,681 · see citing works

The number and distribution of capillaries in muscles with calculations of the oxygen pressure head necessary for supplying the tissue

Krogh, A. (1919) · The Journal of Physiology · doi:10.1113/jphysiol.1919.sp001839 · Cited by 1,433 · see citing works

Hydroxyurea enhances fetal hemoglobin production in sickle cell anemia.

Platt, O., Orkin, S. H., Dover, G., Beardsley, G. P., Miller, B. A., & Nathan, D. G. (1984) · Journal of Clinical Investigation · doi:10.1172/jci111464 · Cited by 532 · see citing works

Kinetics and Mechanism of Deoxyhemoglobin S Gelation: A New Approach to Understanding Sickle Cell Disease

Hofrichter, J., Ross, P. D., & Eaton, W. A. (1974) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.71.12.4864 · Cited by 522 · see citing works

Erythrocyte Adherence to Endothelium in Sickle-Cell Anemia

Hebbel, R. P., Boogaerts, M., Eaton, J. W., & Steinberg, M. H. (1980) · New England Journal of Medicine · doi:10.1056/nejm198005013021803 · Cited by 518 · see citing works

Kinetics of sickle hemoglobin polymerization

Ferrone, F. A., Hofrichter, J., & Eaton, W. A. (1985) · Journal of Molecular Biology · doi:10.1016/0022-2836(85)90175-5 · Cited by 505 · see citing works

Abnormal Adherence of Sickle Erythrocytes to Cultured Vascular Endothelium

Hebbel, R. P., Yamada, O., Moldow, C. F., Jacob, H. S., White, J. G., & Eaton, J. W. (1980) · Journal of Clinical Investigation · doi:10.1172/jci109646 · Cited by 421 · see citing works

Abnormal rheology of oxygenated blood in sickle cell anemia

Chien, S., Usami, S., & Bertles, J. F. (1970) · Journal of Clinical Investigation · doi:10.1172/jci106273 · Cited by 358 · see citing works

Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia?

Powars, D., Weiss, J., Chan, L., & Schroeder, W. (1984) · Blood · doi:10.1182/blood.v63.4.921.921 · Cited by 314 · see citing works

Hemoglobin as a receptor of drugs and peptides: x-ray studies of the stereochemistry of binding

Perutz, M. F., Fermi, G., Abraham, D. J., Poyart, C., & Bursaux, E. (1986) · Journal of the American Chemical Society · doi:10.1021/ja00265a036 · Cited by 299 · see citing works

Kinetics of sickle hemoglobin polymerization

Ferrone, F. A., Hofrichter, J., & Eaton, W. A. (1985) · Journal of Molecular Biology · doi:10.1016/0022-2836(85)90174-3 · Cited by 288 · see citing works

Treatment of sickle cell anemia with 5-azacytidine results in increased fetal hemoglobin production and is associated with nonrandom hypomethylation of DNA around the gamma-delta-beta-globin gene complex.

Charache, S., Dover, G., Smith, K. D., Talbot, C. C., Moyer, M. A., & Boyer, S. H. (1983) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.80.15.4842 · Cited by 270 · see citing works

Irreversibly sickled erythrocytes: a consequence of the heterogeneous distribution of hemoglobin types in sickle-cell anemia

Bertles, J. F., & Milner, P. F. A. (1968) · Journal of Clinical Investigation · doi:10.1172/jci105863 · Cited by 261 · see citing works

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

Ley, T. J., DeSimone, J., Noguchi, C., Turner, P., Schechter, A. N., Heller, P., & Nienhuis, A. (1983) · Blood · doi:10.1182/blood.v62.2.370.370 · Cited by 249 · see citing works

Sickle-Cell Anemia: Molecular and Cellular Bases of Therapeutic Approaches

Dean, J., & Schechter, A. N. (1978) · New England Journal of Medicine · doi:10.1056/nejm197810052991405 · Cited by 237 · see citing works

Static and dynamic rigidities of normal and sickle erythrocytes. Major influence of cell hemoglobin concentration.

Evans, E., Mohandas, N., & Leung, A. (1984) · Journal of Clinical Investigation · doi:10.1172/jci111234 · Cited by 228 · see citing works

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

Kaul, D. K., Fabry, M. E., Windisch, P., Baez, S., & Nagel, R. L. (1983) · Journal of Clinical Investigation · doi:10.1172/jci110960 · Cited by 226 · see citing works

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

Mozzarelli, A., Hofrichter, J., & Eaton, W. A. (1987) · Science · doi:10.1126/science.3603036 · Cited by 222 · see citing works

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

Noguchi, C., & Schechter, A. N. (1981) · Blood · doi:10.1182/blood.v58.6.1057.1057 · Cited by 219 · see citing works

Supersaturation in sickle cell hemoglobin solutions.

Hofrichter, J., Ross, P. D., & Eaton, W. A. (1976) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.73.9.3035 · Cited by 202 · see citing works

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

Brittenham, G., Schechter, A., & Noguchi, C. (1985) · Blood · doi:10.1182/blood.v65.1.183.183 · Cited by 197 · see citing works

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

Nagel, R. L., Bookchin, R. M., Johnson, J., Labie, D., Wajcman, H., Isaac-Sodeye, W. A., Honig, G. R., Schilirò, G., Crookston, J. H., & Matsutomo, K. (1979) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.76.2.670 · Cited by 196 · see citing works

[16] Polarized absorption and linear dichroism spectroscopy of hemoglobin

Eaton, W. A., & Hofrichter, J. (1981) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/0076-6879(81)76126-3 · Cited by 195 · see citing works

Capillary recruitment in exercise: rate, extent, uniformity, and relation to blood flow

Honig, C. R., Odoroff, C. L., & Frierson, J. L. (1980) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.1980.238.1.h31 · Cited by 191 · see citing works

Studies on the Destruction of Red Blood Cells. VIII. Molecular Orientation in Sickle Cell Hemoglobin Solutions.

Harris, J. W. (1950) · Experimental Biology and Medicine · doi:10.3181/00379727-75-18144 · Cited by 190 · see citing works

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

Seakins, M., Gibbs, W. N., Milner, P. F. A., & Bertles, J. F. (1973) · Journal of Clinical Investigation · doi:10.1172/jci107199 · Cited by 185 · see citing works

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

Bishop, M. F., & Ferrone, F. A. (1984) · Biophysical Journal · doi:10.1016/s0006-3495(84)84062-x · Cited by 180 · see citing works

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

Eaton, W., Hofrichter, J., & Ross, P. (1976) · Blood · doi:10.1182/blood.v47.4.621.bloodjournal474621 · Cited by 168 · see citing works

Three-dimensional reconstruction of the fibres of sickle cell haemoglobin

Dykes, G. W., Crepeau, R. H., & Edelstein, S. J. (1978) · Nature · doi:10.1038/272506a0 · Cited by 166 · see citing works

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

Steinberg, M. H., Rosenstock, W., Coleman, M. B., Adams, J. D., Platica, O., Cedeno, M., Rieder, R. F., Wilson, J., Milner, P., & West, S. (1984) · Blood · doi:10.1182/blood.v63.6.1353.1353 · Cited by 161 · see citing works

Thermodynamics of gelation of sickle cell deoxyhemoglobin

Ross, P. D., Hofrichter, J., & Eaton, W. A. (1977) · Journal of Molecular Biology · doi:10.1016/0022-2836(77)90093-6 · Cited by 158 · see citing works

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

Mohandas, N., & Evans, E. (1984) · Blood · doi:10.1182/blood.v64.1.282.282 · Cited by 156 · see citing works

Ligand-induced conformational dependence of hemoglobin in sickling interactions

Bookchin, R. M., & Nagel, R. L. (1971) · Journal of Molecular Biology · doi:10.1016/0022-2836(71)90292-0 · Cited by 151 · see citing works

Periodic Microcirculatory Flow in Patients with Sickle-Cell Disease

Rodgers, G. P., Schechter, A. N., Noguchi, C. T., Klein, H. G., Nienhuis, A. W., & Bonner, R. F. (1984) · New England Journal of Medicine · doi:10.1056/nejm198412133112403 · Cited by 149 · see citing works

Requirements for therapeutic inhibition of sickle haemoglobin gelation

Sunshine, H. R., Hofrichter, J., & Eaton, W. A. (1978) · Nature · doi:10.1038/275238a0 · Cited by 139 · see citing works

Properties of sickle-cell haemoglobin

ALLISON, A. C. (1957) · Biochemical Journal · doi:10.1042/bj0650212 · Cited by 137 · see citing works

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

Bertles, J. F., Rabinowitz, R., & Döbler, J. (1970) · Science · doi:10.1126/science.169.3943.375 · Cited by 135 · see citing works

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

Adachi, K., & Asakura, T. (1979) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)36013-7 · Cited by 124 · see citing works

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

Dover, G. J., Humphries, R. K., Moore, J., Ley, T. J., Young, N. S., Charache, S., & Nienhuis, A. (1986) · Blood · doi:10.1182/blood.v67.3.735.735 · Cited by 123 · see citing works

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 · see citing works

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

Rosa, R. M., Bierer, B. E., Thomas, R., Stoff, J. S., Kruskall, M. S., Robinson, S., Bunn, H. F., & Epstein, F. H. (1980) · New England Journal of Medicine · doi:10.1056/nejm198011133032002 · Cited by 117 · see citing works

Oxygen binding by sickle cell hemoglobin polymers

Sunshine, H. R., Hofrichter, J., Ferrone, F. A., & Eaton, W. A. (1982) · Journal of Molecular Biology · doi:10.1016/0022-2836(82)90432-6 · Cited by 114 · see citing works

Asymmetrical hemoglobin hybrids. Approach to the study of subunit interactions

Bunn, H. F., & McDonough, M. (1974) · Biochemistry · doi:10.1021/bi00702a024 · Cited by 114 · see citing works

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 · see citing works

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

Fabry, M. E., Mears, J., Patel, P. J., Schaefer-Rego, K. E., Carmichael, L., Martinez, G., & Nagel, R. L. (1984) · Blood · doi:10.1182/blood.v64.5.1042.1042 · Cited by 112 · see citing works

LINEAR DICHROISM OF BIOLOGICAL CHROMOPHORES

Hofrichter, J., & Eaton, W. A. (1976) · Annual Review of Biophysics and Bioengineering · doi:10.1146/annurev.bb.05.060176.002455 · Cited by 112 · see citing works

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

Fabry, M. E., & Nagel, R. L. (1982) · PubMed · Cited by 111 · see citing works

Hemolysis in Sickle Cell Disease

Bensinger, T. A. (1974) · Archives of Internal Medicine · doi:10.1001/archinte.1974.00320160118010 · Cited by 110 · see citing works

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 · see citing works

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

Kaul, D., Fabry, M. E., & Nagel, R. (1986) · Blood · doi:10.1182/blood.v68.5.1162.1162 · Cited by 106 · see citing works

Intrinsic microvascular control of tissue oxygen delivery

Granger, H. J., & Shepherd, A. P. (1973) · Microvascular Research · doi:10.1016/s0026-2862(73)80006-8 · Cited by 102 · see citing works

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

Hebbel, R., Moldow, C., & Steinberg, M. (1981) · Blood · doi:10.1182/blood.v58.5.947.947 · Cited by 100 · see citing works

Kinetics of sickle haemoglobin polymerization in single red cells

Coletta, M., Hofrichter, J., Ferrone, F. A., & Eaton, W. A. (1982) · Nature · doi:10.1038/300194a0 · Cited by 96 · see citing works

Irreversibly sickled cells and red cell survival in sickle cell anemia

McCurdy, P. R., & Sherman, A. S. (1978) · The American Journal of Medicine · doi:10.1016/0002-9343(78)90053-0 · Cited by 96 · see citing works

β-Chain contact sites in the haemoglobin S polymer

Nagel, R. L., Johnson, J. E., Bookchin, R. M., Garel, M. C., Rosa, J., Schilirò, G., Wajcman, H., Labie, D., Moo-Penn, W. F., & Castro, O. L. (1980) · Nature · doi:10.1038/283832a0 · Cited by 95 · see citing works

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

Padlan, E. A., & Love, W. E. (1982) · Worldwide Protein Data Bank · doi:10.2210/pdb1hbs/pdb · Cited by 95 · see citing works

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

Malfa, R., & Steinhardt, J. (1974) · Biochemical and Biophysical Research Communications · doi:10.1016/s0006-291x(74)80062-8 · Cited by 93 · see citing works

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

Mears, J., Lachman, H., Labie, D., & Nagel, R. L. (1983) · Blood · doi:10.1182/blood.v62.2.286.bloodjournal622286 · Cited by 93 · see citing works

Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.

Noguchi, C. T., Torchia, D. A., & Schechter, A. N. (1983) · Journal of Clinical Investigation · doi:10.1172/jci111055 · Cited by 92 · see citing works

Hemodynamic Studies in Sickle Cell Anemia

Leight, L., Snider, T. H., Clifford, G. O., & Hellems, H. K. (1954) · Circulation · doi:10.1161/01.cir.10.5.653 · Cited by 92 · see citing works

Thermodynamic studies of polymerization of deoxygenated sickle cell hemoglobin.

Magdoff-Fairchild, B., Poillon, W. N., Li, T., & Bertles, J. F. (1976) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.73.4.990 · Cited by 92 · see citing works

The rates of polymerization and depolymerization of sickle cell hemoglobin

Moffat, K., & Gibson, Q. H. (1974) · Biochemical and Biophysical Research Communications · doi:10.1016/0006-291x(74)90558-0 · Cited by 90 · see citing works

Cardiac function in sickle cell anemia

Denenberg, B. S., Criner, G. J., Jones, R. A., & Spann, J. F. (1983) · The American Journal of Cardiology · doi:10.1016/0002-9149(83)90208-4 · Cited by 90 · see citing works

Calorimetric and optical characterization of sickle cell hemoglobin gelation

Ross, P. D., Hofrichter, J., Eaton, W. A., & Hofrichter, J. (1975) · Journal of Molecular Biology · doi:10.1016/0022-2836(75)90345-9 · Cited by 89 · see citing works

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

Ceulaer, K. D., Higgs, D. R., Weatherall, D. J., Hayes, R., Serjeant, B. E., & Serjeant, G. R. (1983) · New England Journal of Medicine · doi:10.1056/nejm198307213090320 · Cited by 89 · see citing works

Circulatory dynamics at rest and on exercise in the hyperkinetic states.

Jm, B., Donald, K. W., & WADE, O. L. (1955) · PubMed · Cited by 89 · see citing works

Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

Noguchi, C. T., Torchia, D. A., & Schechter, A. N. (1980) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.77.9.5487 · Cited by 88 · see citing works

Tank Tread Motion of Red Cell Membranes in Viscometric Flow: Behavior of Intracellular and Extracellular Markers (with Film)

Fischer, T., & Schmid-Schönbein, H. (1978) · Springer eBooks · doi:10.1007/978-3-642-67059-6_26 · Cited by 88 · see citing works

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

Sproule, B. J., Halden, E. R., & Miller, W. F. (1958) · Journal of Clinical Investigation · doi:10.1172/jci103629 · Cited by 83 · see citing works

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

Nash, G. B., Johnson, C., & Meiselman, H. J. (1986) · Blood · doi:10.1182/blood.v67.1.110.110 · Cited by 82 · see citing works

Physiological and x-ray studies of potential antisickling agents.

Abraham, D. J., Perutz, M. F., & Phillips, S. E. V. (1983) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.80.2.324 · Cited by 82 · see citing works

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

Billett, H., Kim, K., Fabry, M. E., & Nagel, R. L. (1986) · Blood · doi:10.1182/blood.v68.1.301.301 · Cited by 78 · see citing works

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

Steinberg, M. H., & Hebbel, R. P. (1983) · American Journal of Hematology · doi:10.1002/ajh.2830140412 · Cited by 75 · see citing works

Molecular topology in crystals and fibers of hemoglobin S

Edelstein, S. J. (1981) · Journal of Molecular Biology · doi:10.1016/0022-2836(81)90381-8 · Cited by 72 · see citing works

Solubilization of hemoglobin S by other hemoglobins.

Benesch, R., Edalji, R. P., Benesch, R., & Kwong, S. (1980) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.77.9.5130 · Cited by 69 · see citing works

Cardiorespiratory adjustments in chronic sickle cell anemia.

Lonsdorfer, J., Bogui, P., Otayeck, A., Bursaux, E., Poyart, C., & Cabannes, R. (1983) · PubMed · Cited by 69 · see citing works

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

Pumphrey, J. G., & Steinhardt, J. (1977) · Journal of Molecular Biology · doi:10.1016/s0022-2836(77)80187-3 · Cited by 67 · see citing works

Ligand binding and the gelation of sickle cell hemoglobin

Hofrichter, J. (1979) · Journal of Molecular Biology · doi:10.1016/0022-2836(79)90092-5 · Cited by 66 · see citing works

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

Dover, G. J., Charache, S., Boyer, S. H., Vogelsang, G. B., & Moyer, M. A. (1985) · Blood · doi:10.1182/blood.v66.3.527.527 · Cited by 66 · see citing works

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

Harris, J. W., & Bensusan, H. B. (1975) · PubMed · Cited by 66 · see citing works

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 · see citing works

Concerted Formation of the Gel of Hemoglobin S

Williams, R. C. (1973) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.70.5.1506 · Cited by 62 · see citing works

Electrostatic attraction governs the dimer assembly of human hemoglobin.

Mrabet, N. T., McDonald, M. J., Turci, S. M., Sarkar, R., Szabó, A., & Bunn, H. F. (1986) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)89237-2 · Cited by 59 · see citing works

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

Noguchi, C. T., & Schechter, A. N. (1977) · Biochemical and Biophysical Research Communications · doi:10.1016/0006-291x(77)90350-3 · Cited by 58 · see citing works

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

Miller, D. M., Winslow, R. M., Hg, K., Wilson, K., Brown, F. L., & Statham, N. J. (1980) · Blood · doi:10.1182/blood.v56.6.1127.1127 · Cited by 57 · see citing works

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

Asakura, T., & Mayberry, J. C. (1984) · PubMed · Cited by 55 · see citing works

Oxygen binding to sickle cell hemoglobin

Gill, S. J., Benedict, R. C., Fall, L., Spokane, R. B., & Wyman, J. (1979) · Journal of Molecular Biology · doi:10.1016/0022-2836(79)90425-x · Cited by 55 · see citing works

In vivo study of the sickle cell phenomenon.

Wn, J., Rucknagel, D., & Wj, T. (1960) · PubMed · Cited by 54 · see citing works

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

Poillon, W. N., & Bertles, J. F. (1979) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)50782-1 · Cited by 53 · see citing works

Calculated dispersion of capillary transit times: significance for oxygen exchange

Honig, C. R., & Odoroff, C. L. (1981) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.1981.240.2.h199 · Cited by 53 · see citing works

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

Pumphrey, J. G., & Steinhardt, J. (1976) · Biochemical and Biophysical Research Communications · doi:10.1016/s0006-291x(76)80278-1 · Cited by 50 · see citing works

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

Mr, C., Mohandas, N., Embury, S., & Lubin, B. H. (1982) · Blood · doi:10.1182/blood.v60.3.659.659 · Cited by 49 · see citing works

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 · see citing works

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 46 · see citing works

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

Kowalczykowski, S., & Steinhardt, J. (1977) · Journal of Molecular Biology · doi:10.1016/0022-2836(77)90097-3 · Cited by 46 · see citing works

Sickling times of individual erythrocytes at zero Po2.

Zarkowsky, H. S., & Hochmuth, R. M. (1975) · Journal of Clinical Investigation · doi:10.1172/jci108149 · Cited by 45 · see citing works

Cardiovascular findings in children with sickle cell anemia

Shubin, H., Kaufman, R., Shapiro, M. J., & Levinson, D. C. (1960) · The American Journal of Cardiology · doi:10.1016/0002-9149(60)90287-3 · Cited by 45 · see citing works

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

Walder, J. A., Zaugg, R. H., Iwaoka, R. S., Watkin, W. G., & Klotz, I. M. (1977) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.74.12.5499 · Cited by 43 · see citing works

THE ALVEOLAR-ARTERIAL OXYGEN PRESSURE GRADIENT IN ANEMIA 1

Ryan, J. M., & Hickam, J. B. (1952) · Journal of Clinical Investigation · doi:10.1172/jci102589 · Cited by 43 · see citing works

Lung function in children with sickle cell anemia.

Ma, W., Platt, O., & Dj, S. (1979) · PubMed · doi:10.1164/arrd.1979.120.1.210 · Cited by 39 · see citing works

Comparative evaluation of fifteen anti-sickling agents

Chang, H., Ewert, S. M., Bookchin, R. M., & Nagel, R. L. (1983) · Blood · doi:10.1182/blood.v61.4.693.693 · Cited by 38 · see citing works

KINETICS OF GAS TRANSPORT IN THE BLOOD

Roughton, F. J. W. (1963) · British Medical Bulletin · doi:10.1093/oxfordjournals.bmb.a070013 · Cited by 38 · see citing works

Oxygen Transport in Sickle Cell Anemia

Milner, P. (1974) · Archives of Internal Medicine · doi:10.1001/archinte.1974.00320160059006 · Cited by 37 · see citing works

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

Moffat, K. (1974) · Science · doi:10.1126/science.185.4147.274 · Cited by 37 · see citing works

Ultrastructure of Sickling and Unsickling in Time‐Lapse Studies

HAHN, J. A., MESSER, M. J., & Bradley, T. B. (1976) · British Journal of Haematology · doi:10.1111/j.1365-2141.1976.tb03601.x · Cited by 37 · see citing works

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

Kaul, D. K., Nagel, R. L., & Baez, S. (1983) · Microvascular Research · doi:10.1016/0026-2862(83)90068-7 · Cited by 37 · see citing works

ARTERIAL BLOOD OXYGENATION IN SICKLE CELL ANEMIA*

Fowler, N. O., Smith, O., & Greenfield, J. C. (1957) · The American Journal of the Medical Sciences · doi:10.1097/00000441-195710000-00010 · Cited by 37 · see citing works

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

Adachi, K., & Asakura, T. (1978) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)37961-9 · Cited by 36 · see citing works

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

Gulley, M. L., Ross, D. W., Féo, C., & Orringer, E. P. (1982) · American Journal of Hematology · doi:10.1002/ajh.2830130403 · Cited by 34 · see citing works

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

Minton, A. P. (1975) · Journal of Molecular Biology · doi:10.1016/0022-2836(75)90396-4 · Cited by 34 · see citing works

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

Briehl, R. W. (1978) · Journal of Molecular Biology · doi:10.1016/0022-2836(78)90205-x · Cited by 32 · see citing works

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

Charache, S., & Wg, W. (1981) · Blood · doi:10.1182/blood.v58.5.892.892 · Cited by 32 · see citing works

The effect of erythrocyte membrane preparations on the polymerization of sickle hemoglobin.

Goldberg, M. A., Lalos, A. T., & Bunn, H. F. (1981) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)70118-5 · Cited by 31 · see citing works

Carbon-13-proton nuclear magnetic double-resonance study of deoxyhemoglobin S gelation

Sutherland, J. W. H., Egan, W., Schechter, A. N., & Torchia, D. A. (1979) · Biochemistry · doi:10.1021/bi00576a025 · Cited by 31 · see citing works

The many pathophysiologies of sickle cell anemia

Nagel, R. L., & Fabry, M. E. (1985) · American Journal of Hematology · doi:10.1002/ajh.2830200216 · Cited by 28 · see citing works

Kinetics of the polymerization of hemoglobin in high and low phosphate buffers.

Adachi, K., & Asakura, T. (1982) · PubMed · Cited by 27 · see citing works

Mixed gelation theory

Behe, M. J., & Englander, S. W. (1979) · Journal of Molecular Biology · doi:10.1016/0022-2836(79)90254-7 · Cited by 25 · see citing works

Capillary Flow: History, experiments and theory

Skalak, R., & Chien, S. (1981) · Biorheology · doi:10.3233/bir-1981-183-602 · Cited by 25 · see citing works

Inhibition of erythrocyte sickling by thiol reagents.

Garel, M. C., Domenget, C., Galactéros, F., Martin, J., & Beuzard, Y. (1984) · Molecular Pharmacology · doi:10.1016/s0026-895x(25)15109-2 · Cited by 25 · see citing works

Red cell membrane abnormalities in sickle cell anemia.

Bookchin, R. M., & Vl, L. (1983) · PubMed · Cited by 24 · see citing works

Ligand kinetics of hemoglobin S containing erythrocytes.

Harrington, J. P., Elbaum, D., Bookchin, R. M., Wittenberg, J. B., & Nagel, R. L. (1977) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.74.1.203 · Cited by 23 · see citing works

The in vivo sickle phenomenon: a reappraisal.

Serjeant, G. R., Petch, M. C., & Serjeant, B. E. (1973) · PubMed · Cited by 23 · see citing works

Arterial oxygen unsaturation in sickle cell disease.

Bromberg, P. A., & Jensen, W. N. (1967) · PubMed · doi:10.1164/arrd.1967.96.3.400 · Cited by 22 · see citing works

The Rate of Sickling of Cells Containing Sickle-Cell Haemoglobin

RAMPLING, M. W., & Sirs, J. A. (1973) · Clinical Science · doi:10.1042/cs0450655 · Cited by 22 · see citing works

Antisickling effect of tellurite: a potent membrane-acting agent in vitro

Asakura, T., Shibutani, Y., Reilly, M., & DeMeio, R. H. (1984) · Blood · doi:10.1182/blood.v64.1.305.305 · Cited by 19 · see citing works

Kinetics of Hb S gelation Effect of alkylureas, ionic strength and other hemoglobins

Elbaum, D., Harrington, J. P., Bookchin, R. M., & Nagel, R. L. (1978) · Biochimica et Biophysica Acta (BBA) - Protein Structure · doi:10.1016/0005-2795(78)90005-3 · Cited by 18 · see citing works

The Interaction of Sickle Hemoglobin with DPG, CO2 and with Other Hemoglobins: Formation of Asymmetrical Hybrids

Bunn, H. F. (1972) · Advances in experimental medicine and biology · doi:10.1007/978-1-4684-3222-0_3 · Cited by 17 · see citing works

Oxygen Delivery to Muscle Cells during Capillary Occlusion by Sickled Erythrocytes

LaCelle, P. L. (1978) · Springer eBooks · doi:10.1007/978-3-642-67059-6_21 · Cited by 15 · see citing works

A model of microvascular oxygen transport in sickle cell disease

Vayo, M. M., Lipowsky, H. H., Karp, N. S., Schmalzer, E. A., & Chein, S. (1985) · Microvascular Research · doi:10.1016/0026-2862(85)90050-0 · Cited by 14 · see citing works

Multiple nature of polymers of deoxyhemoglobin S prepared by different methods.

Adachi, K., & Asakura, T. (1983) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)32827-8 · Cited by 12 · see citing works

Polarity of the 14-strand fibers of sickle cell hemoglobin determined by cross-correlation methods

Crepeau, R. H., & Edelstein, S. J. (1984) · Ultramicroscopy · doi:10.1016/0304-3991(84)90052-4 · Cited by 12 · see citing works

Saturation transfer electron paramagnetic resonance detection of sickle hemoglobin aggregation during deoxygenation

Thiyagarajan, P., & Johnson, M. E. (1983) · Biophysical Journal · doi:10.1016/s0006-3495(83)84394-x · Cited by 11 · see citing works

HEMOGLOBIN INTERACTIONS AND WHOLE BLOOD OXYGEN EQUILIBRIUM CURVES IN SICKLING DISORDERS

Winslow, R. M. (1978) · Elsevier eBooks · doi:10.1016/b978-0-12-164350-8.50031-5 · Cited by 11 · see citing works

Formation of nuclei during delay time prior to aggregation of deoxyhemoglobin s in concentrated phosphate buffer

Adachi, K., Asakura, T., & McConnell, M. L. (1979) · Biochimica et Biophysica Acta (BBA) - Protein Structure · doi:10.1016/0005-2795(79)90152-1 · Cited by 8 · see citing works

Kinetics of oxygenation and deoxygenation of erythrocytes containing hemoglobin S

Rotman, H. H., Klocke, R. A., Andersson, K. K., D’Alecy, L. G., & Forster, R. E. (1974) · Respiration Physiology · doi:10.1016/0034-5687(74)90003-6 · Cited by 6 · see citing works

Polymerization of carbamylated deoxyhemoglobin S in concentrated phosphate buffer.

Ip, C. Y., Asakura, T., & Adachi, K. (1982) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)33593-2 · Cited by 5 · see citing works

EFFECTORS OF THE RATE OF DEOXYHEMOGLOBIN S POLYMERIZATION11The research upon which this publication is based was performed pursuant to Contract No. N01-HB-2-2954 with the National Institutes of Health, Department of Health, Education, and Welfare.

Cottam, G. L., Shibata, K., & Waterman, M. R. (1978) · Elsevier eBooks · doi:10.1016/b978-0-12-164350-8.50056-x · Cited by 4 · see citing works

Viscometric and spectrophotometric measurements of hemoglobin S polymerization kinetics

Wenger, G., & Sp, B. (1984) · Blood · doi:10.1182/blood.v63.4.897.897 · Cited by 4 · see citing works

Effect of other hemoglobins on gelation of sickle cell hemoglobin.

Sunshine, H. R. (1982) · PubMed · Cited by 3 · see citing works

Experimentally-Induced Alterations in the Kinetics of Erythrocyte Sickling

Zarkowsky, H. S., & Hochmuth, R. M. (1978) · Springer eBooks · doi:10.1007/978-3-642-67059-6_23 · Cited by 3 · see citing works

Effects of red cell membrane on the polymerization of sickle hemoglobin.

Goldberg, M. A., Lalos, A. T., Himmelstein, B., & Bunn, H. F. (1982) · PubMed · Cited by 3 · see citing works

Oxygen transport and the sickle cell.

Nagel, R. L., & Bookchin, R. M. (1981) · PubMed · Cited by 2 · see citing works

Functional significance of cell volume regulatory mechanisms

LANG, F., BUSCH, G. L., RITTER, M., VÖLKL, H., WALDEGGER, S., GULBINS, E., & HÄUSSINGER, D. (1998) · Physiological Reviews · doi:10.1152/physrev.1998.78.1.247 · Cited by 1,891 · see citing works

Pathogenesis and Treatment of Sickle Cell Disease

Bunn, H. F. (1997) · New England Journal of Medicine · doi:10.1056/nejm199709113371107 · Cited by 1,154 · see citing works

A new era for understanding amyloid structures and disease

Iadanza, M. G., Jackson, M. P., Hewitt, E. W., Ranson, N. A., & Radford, S. E. (2018) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-018-0060-8 · Cited by 969 · see citing works

Sickle Cell Hemoglobin Polymerization

Eaton, W. A., & Hofrichter, J. (1990) · Advances in protein chemistry · doi:10.1016/s0065-3233(08)60287-9 · Cited by 623 · see citing works

Fetal hemoglobin in sickle cell anemia

Akinsheye, I., Alsultan, A., Solovieff, N., Ngo, D., Baldwin, C. T., Sebastiani, P., Chui, D. H. K., & Steinberg, M. H. (2011) · Blood · doi:10.1182/blood-2011-03-325258 · Cited by 553 · see citing works

Membrane Mechanisms and Intracellular Signalling in Cell Volume Regulation

Hoffmann, E. K., & Dunham, P. B. (1995) · International review of cytology · doi:10.1016/s0074-7696(08)62498-5 · Cited by 469 · see citing works

Inhibition of Ca(2+)-dependent K+ transport and cell dehydration in sickle erythrocytes by clotrimazole and other imidazole derivatives.

Brugnara, C., Franceschi, L. D., & Alper, S. L. (1993) · Journal of Clinical Investigation · doi:10.1172/jci116597 · Cited by 359 · see citing works

Chronic Renal Failure in Sickle Cell Disease: Risk Factors, Clinical Course, and Mortality

Powars, D. R., Elliott-Mills, D. D., Chan, L. S., Niland, J. C., Hiti, A. L., Opas, L. M., & Johnson, C. S. (1991) · Annals of Internal Medicine · doi:10.7326/0003-4819-115-8-614 · Cited by 357 · see citing works

Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions

Kato, G. J., Hebbel, R. P., Steinberg, M. H., & Gladwin, M. T. (2009) · American Journal of Hematology · doi:10.1002/ajh.21475 · Cited by 349 · see citing works

Hydroxyurea and Sickle Cell Anemia Clinical Utility of a Myelosuppressive “Switching” Agent

Charache, S., Barton, F. B., Moore, R. D., Terrin, M. L., Steinberg, M. H., Dover, G. J., Ballas, S. K., McMahon, R. P., Castro, O. L., & Orringer, E. P. (1996) · Medicine · doi:10.1097/00005792-199611000-00002 · Cited by 335 · see citing works

Correction of sickle cell disease by homologous recombination in embryonic stem cells

Wu, L.-C., Sun, C., Ryan, T. M., Pawlik, K. M., Ren, J., & Townes, T. M. (2006) · Blood · doi:10.1182/blood-2006-02-004812 · Cited by 327 · see citing works

Beyond hemoglobin polymerization: the red blood cell membrane and sickle disease pathophysiology

Hebbel, R. (1991) · Blood · doi:10.1182/blood.v77.2.214.bloodjournal772214 · Cited by 325 · see citing works

Knockout-Transgenic Mouse Model of Sickle Cell Disease

Ryan, T. M., Ciavatta, D. J., & Townes, T. M. (1997) · Science · doi:10.1126/science.278.5339.873 · Cited by 300 · see citing works

The functional nitrite reductase activity of the heme-globins

Gladwin, M. T., & Kim-Shapiro, D. B. (2008) · Blood · doi:10.1182/blood-2008-01-115261 · Cited by 293 · see citing works

Alpha 4 beta 1-integrin expression on sickle reticulocytes: vascular cell adhesion molecule-1-dependent binding to endothelium

Swerlick, R., Eckman, J., Kumar, A. F., Jeitler, M., & Wick, T. (1993) · Blood · doi:10.1182/blood.v82.6.1891.1891 · Cited by 275 · see citing works

Fetal hemoglobin in sickle cell anemia: a glass half full?

Steinberg, M. H., Chui, D. H. K., Dover, G. J., Sebastiani, P., & Alsultan, A. (2014) · Blood · doi:10.1182/blood-2013-09-528067 · Cited by 267 · see citing works

Treating sickle cell disease by targeting HbS polymerization

Eaton, W. A., & Bunn, H. F. (2017) · Blood · doi:10.1182/blood-2017-02-765891 · Cited by 255 · see citing works

Vascular occlusion in sickle cell disease: current concepts and unanswered questions

Francis, R. J., & Johnson, C. (1991) · Blood · doi:10.1182/blood.v77.7.1405.bloodjournal7771405 · Cited by 242 · see citing works

Therapy with oral clotrimazole induces inhibition of the Gardos channel and reduction of erythrocyte dehydration in patients with sickle cell disease.

Brugnara, C., Gee, B., Armsby, C. C., Kurth, S., Sakamoto, M., Rifai, N., Alper, S. L., & Platt, O. (1996) · Journal of Clinical Investigation · doi:10.1172/jci118537 · Cited by 231 · see citing works

Rails and anchors: guiding and trapping droplet microreactors in two dimensions

Abbyad, P., Dangla, R., Alexandrou, A., & Baroud, C. N. (2010) · Lab on a Chip · doi:10.1039/c0lc00104j · Cited by 225 · see citing works

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

Mozzarelli, A., Hofrichter, J., & Eaton, W. A. (1987) · Science · doi:10.1126/science.3603036 · Cited by 222 · see citing works

Sickle Red Cell–Endothelium Interactions

KAUL, D. K., FINNEGAN, E., & BARABINO, G. A. (2009) · Microcirculation · doi:10.1080/10739680802279394 · Cited by 208 · see citing works

ICA-17043, a novel Gardos channel blocker, prevents sickled red blood cell dehydration in vitro and in vivo in SAD mice

Stocker, J. W., Franceschi, L. D., McNaughton-Smith, G. A., Corrocher, R., Beuzard, Y., & Brugnara, C. (2003) · Blood · doi:10.1182/blood-2002-05-1433 · Cited by 205 · see citing works

Correction of a mouse model of sickle cell disease: lentiviral/antisickling beta-globin gene transduction of unmobilized, purified hematopoietic stem cells.

Levasseur, D., Ryan, T. M., Pawlik, K. M., & Townes, T. (2003) · Blood · doi:10.1182/blood-2003-04-1251 · Cited by 200 · see citing works

Treatment of Sickle Cell Anemia with Hydroxyurea and Erythropoietin

Goldberg, M. A., Brugnara, C., Dover, G. J., Schapira, L., Charache, S., & Bunn, H. F. (1990) · New England Journal of Medicine · doi:10.1056/nejm199008093230602 · Cited by 193 · see citing works

A multiparameter analysis of sickle erythrocytes in patients undergoing hydroxyurea therapy

Bridges, K., Barabino, G., Brugnara, C., Cho, M., Christoph, G., Dover, G., Ewenstein, B., Golan, D., Guttmann, C., Hofrichter, J., et al. (1996) · Blood · doi:10.1182/blood.v88.12.4701.bloodjournal88124701 · Cited by 184 · see citing works

Supersaturation is a major driving force for protein aggregation in neurodegenerative diseases

Ciryam, P., Kundra, R., Morimoto, R. I., Dobson, C. M., & Vendruscolo, M. (2015) · Trends in Pharmacological Sciences · doi:10.1016/j.tips.2014.12.004 · Cited by 173 · see citing works

Oral magnesium supplements reduce erythrocyte dehydration in patients with sickle cell disease.

Franceschi, L. D., Bachir, D., Galactéros, F., Tchernia, G., Cynober, T., Alper, S. L., Platt, O., Beuzard, Y., & Brugnara, C. (1997) · Journal of Clinical Investigation · doi:10.1172/jci119713 · Cited by 167 · see citing works

Long-Term Engraftment and Fetal Globin Induction upon BCL11A Gene Editing in Bone-Marrow-Derived CD34 + Hematopoietic Stem and Progenitor Cells

Chang, K.-H., Smith, S. E., Sullivan, T., Chen, K., Zhou, Q., West, J. A., Liu, M., Liu, Y., Vieira, B. F., Sun, C., et al. (2017) · Molecular Therapy - Methods & Clinical Development · doi:10.1016/j.omtm.2016.12.009 · Cited by 155 · see citing works

Sickle Cell Disease and Anesthesia

Firth, P. G., Head, C. A., & Warltier, D. C. (2004) · Anesthesiology · doi:10.1097/00000542-200409000-00027 · Cited by 157 · see citing works

Life-Threatening Infectious Complications in Sickle Cell Disease: A Concise Narrative Review

Ochocinski, D., Dalal, M., Black, L. V., Carr, S. B., Lew, J., Sullivan, K., & Kissoon, N. “. (2020) · Frontiers in Pediatrics · doi:10.3389/fped.2020.00038 · Cited by 162 · see citing works

The pathophysiology of vascular obstruction in the sickle syndromes

Kaul, D. K. (1996) · Blood Reviews · doi:10.1016/s0268-960x(96)90018-1 · Cited by 154 · see citing works

Newer Aspects of the Pathophysiology of Sickle Cell Disease Vaso-Occlusion

Conran, N., Franco‐Penteado, C. F., & Costa, F. F. (2009) · Hemoglobin · doi:10.1080/03630260802625709 · Cited by 151 · see citing works

Sickle Cell Vaso-Occlusion

Fabry, M. E., & Kaul, D. K. (1991) · Hematology/Oncology Clinics of North America · doi:10.1016/s0889-8588(18)30421-0 · Cited by 144 · see citing works

Treatment with oral clotrimazole blocks Ca(2+)-activated K+ transport and reverses erythrocyte dehydration in transgenic SAD mice. A model for therapy of sickle cell disease.

Franceschi, L. D., Saadane, N., Trudel, M., Alper, S. L., Brugnara, C., & Beuzard, Y. (1994) · Journal of Clinical Investigation · doi:10.1172/jci117149 · Cited by 142 · see citing works

Low concentrations of nitric oxide increase oxygen affinity of sickle erythrocytes in vitro and in vivo.

Head, C. A., Brugnara, C., Martinez-Ruiz, R., Kacmarek, R. M., Bridges, K. R., Kuter, D. J., Bloch, K. D., & Zapol, W. M. (1997) · Journal of Clinical Investigation · doi:10.1172/jci119631 · Cited by 138 · see citing works

Fetal hemoglobin in sickle cell anemia

Steinberg, M. H. (2020) · Blood · doi:10.1182/blood.2020007645 · Cited by 136 · see citing works

Disorders of erythrocyte hydration

Gallagher, P. G. (2017) · Blood · doi:10.1182/blood-2017-04-590810 · Cited by 133 · see citing works

Sickle‐cell haemoglobin polymerization: is it the primary pathogenic event of sickle‐cell anaemia?

Vekilov, P. G. (2007) · British Journal of Haematology · doi:10.1111/j.1365-2141.2007.06794.x · Cited by 132 · see citing works

Human Sickle Hemoglobin in Transgenic Mice

Ryan, T. M., Townes, T. M., Reilly, M. P., Asakura, T., Palmiter, R. D., Brinster, R. L., & Behringer, R. R. (1990) · Science · doi:10.1126/science.2154033 · Cited by 130 · see citing works

Malaria and red cell genetic defects

Nagel, R. L., & Roth, E. J. (1989) · Blood · doi:10.1182/blood.v74.4.1213.1213 · Cited by 128 · see citing works

Insight into the complex pathophysiology of sickle cell anaemia and possible treatment

Piccin, A., Murphy, C., Eakins, E., Rondinelli, M. B., Daves, M., Vecchiato, C., Wolf, D. G. F., Mahon, C. M., & Smith, O. P. (2019) · European Journal Of Haematology · doi:10.1111/ejh.13212 · Cited by 126 · see citing works

Sickle Red Cell Microrheology and Sickle Blood Rheology

Ballas, S. K., & Mohandas, N. (2004) · Microcirculation · doi:10.1080/10739680490279410 · Cited by 123 · see citing works

Oxygen-Sensitive Membrane Transporters in Vertebrate Red Cells

Gibson, J. S., Cossins, A. R., & Ellory, J. C. (2000) · Journal of Experimental Biology · doi:10.1242/jeb.203.9.1395 · Cited by 123 · see citing works

Kinetics of sickle cell biorheology and implications for painful vasoocclusive crisis

Du, E., Diez-Silva, M., Kato, G. J., Dao, M., & Suresh, S. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1424111112 · Cited by 120 · see citing works

A Biophysical Indicator of Vaso-occlusive Risk in Sickle Cell Disease

Wood, D. K., Soriano, A., Mahadevan, L., Higgins, J. M., & Bhatia, S. N. (2012) · Science Translational Medicine · doi:10.1126/scitranslmed.3002738 · Cited by 120 · see citing works

Metastable Mesoscopic Clusters in Solutions of Sickle-Cell Hemoglobin

Pan, W., Galkin, O., Filobelo, L. F., Nagel, R. L., & Vekilov, P. G. (2006) · Biophysical Journal · doi:10.1529/biophysj.106.094854 · Cited by 119 · see citing works

Gene therapy for sickle cell disease: An update

Demirci, S., Uchida, N., & Tisdale, J. F. (2018) · Cytotherapy · doi:10.1016/j.jcyt.2018.04.003 · Cited by 119 · see citing works

Evolution of allosteric models for hemoglobin

Eaton, W. A., Henry, E. R., Hofrichter, J., Bettati, S., Viappiani, C., & Mozzarelli, A. (2007) · IUBMB Life · doi:10.1080/15216540701272380 · Cited by 114 · see citing works

The Acute Chest Syndrome of Sickle Cell Disease

Platt, O. S. (2000) · New England Journal of Medicine · doi:10.1056/nejm200006223422510 · Cited by 112 · see citing works

β-globin gene transfer to human bone marrow for sickle cell disease

Romero, Z. G., Urbinati, F., Geiger, S., Cooper, A. R., Wherley, J., Kaufman, M. L., Hollis, R. P., de Assín, R. R., Senadheera, S., Sahagian, A., et al. (2013) · Journal of Clinical Investigation · doi:10.1172/jci67930 · Cited by 107 · see citing works

Pathophysiology and therapy for haemoglobinopathies; Part I: sickle cell disease

Madigan, C., & Malik, P. (2006) · Expert Reviews in Molecular Medicine · doi:10.1017/s1462399406010659 · Cited by 106 · see citing works

CRISPR/Cas9 gene editing for curing sickle cell disease

Park, S. H., & Bao, G. (2021) · Transfusion and Apheresis Science · doi:10.1016/j.transci.2021.103060 · Cited by 110 · see citing works

ßs-Gene-Cluster Haplotypes in Sickle Cell Anemia: Clinical and Hematologic Features

Powars, D. R. (1991) · Hematology/Oncology Clinics of North America · doi:10.1016/s0889-8588(18)30426-x · Cited by 104 · see citing works

Double-blind, placebo-controlled, randomised cross-over clinical trial of NIPRISAN? in patients with Sickle Cell Disorder

Wambebe, C. O. (2001) · Phytomedicine · doi:10.1078/0944-7113-00040 · Cited by 104 · see citing works

Subclinical ischaemic episodes during the steady state of sickle cell anaemia.

Akinola, N. O., Stevens, S. M., FRANKLIN, I. M., Nash, G. B., & Stuart, J. (1992) · Journal of Clinical Pathology · doi:10.1136/jcp.45.10.902 · Cited by 98 · see citing works

Polymerization and Sickle Cell Disease: A Molecular View

Ferrone, F. A. (2004) · Microcirculation · doi:10.1080/10739680490278312 · Cited by 98 · see citing works

The Biophysics of Sickle Cell Hydroxyurea Therapy

Eaton, W. A., & Hofrichter, J. (1995) · Science · doi:10.1126/science.7539154 · Cited by 96 · see citing works

Understanding the Shape of Sickled Red Cells

Christoph, G. W., Hofrichter, J., & Eaton, W. A. (2004) · Biophysical Journal · doi:10.1529/biophysj.104.051250 · Cited by 94 · see citing works

Emerging disease-modifying therapies for sickle cell disease

Carden, M. A., & Little, J. A. (2019) · Haematologica · doi:10.3324/haematol.2018.207357 · Cited by 97 · see citing works

Interfacial Adsorption and Aggregation Associated Changes in Secondary Structure of Human Calcitonin Monitored by ATR-FTIR Spectroscopy

Bauer, H. H., Mueller, M., Goette, J., Merkle, H. P., & Fringeli, U. P. (1994) · Biochemistry · doi:10.1021/bi00206a034 · Cited by 93 · see citing works

Spectral domain optical coherence tomography in patients with sickle cell disease

Mathew, R., Bafiq, R., Ramu, J., Pearce, E., Richardson, M., Drašar, E., Thein, S. L., & Sivaprasad, S. (2015) · British Journal of Ophthalmology · doi:10.1136/bjophthalmol-2014-305532 · Cited by 91 · see citing works

Treatment of sickle cell disease by increasing oxygen affinity of hemoglobin

Henry, E. R., Metaferia, B. B., Li, Q., Delenko, J., Best, R. B., Glass, K. E., Cellmer, T., Dunkelberger, E. B., Conrey, A. K., Thein, S. L., et al. (2021) · Blood · doi:10.1182/blood.2021012070 · Cited by 91 · see citing works

A second generation transgenic mouse model expressing both hemoglobin S (HbS) and HbS-Antilles results in increased phenotypic severity

Fabry, M. E., Sengupta, A., Suzuka, S. M., Costantini, F. D., Rubin, E. M., Hofrichter, J., Christoph, G. W., Manci, E. A., Culberson, D., & Factor, S. M. (1995) · Blood · doi:10.1182/blood.v86.6.2419.bloodjournal8662419 · Cited by 90 · see citing works

A Systems Biology Consideration of the Vasculopathy of Sickle Cell Anemia: The Need for Multi-Modality Chemo-Prophylaxis

Hebbel, R. P., Vercellotti, G. M., & Nath, K. A. (2009) · Cardiovascular & Haematological Disorders - Drug Targets · doi:10.2174/1871529x10909040271 · Cited by 89 · see citing works

Genetic epidemiology of the βs gene

Nagel, R. L., & Fleming, A. F. (1992) · Baillière s Clinical Haematology · doi:10.1016/s0950-3536(11)80023-5 · Cited by 87 · see citing works

The Fibrillary Glomerulopathies

Korbet, S. M., Schwartz, M. M., & Lewis, E. J. (1994) · American Journal of Kidney Diseases · doi:10.1016/s0272-6386(12)70290-1 · Cited by 87 · see citing works

Hemoglobin variants and activity of the (K+Cl-) cotransport system in human erythrocytes

Olivieri, O., Vitoux, D., Galactéros, F., Bachir, D., Blouquit, Y., Beuzard, Y., & Brugnara, C. (1992) · Blood · doi:10.1182/blood.v79.3.793.793 · Cited by 86 · see citing works

Differential oxygen sensitivity of the K+‐Cl− cotransporter in normal and sickle human red blood cells

Gibson, J. S., Speake, P. F., & Ellory, J. C. (1998) · The Journal of Physiology · doi:10.1111/j.1469-7793.1998.225bi.x · Cited by 84 · see citing works

Management of Pregnancy in Sickle Cell Syndromes

Koshy, M., & Burd, L. I. (1991) · Hematology/Oncology Clinics of North America · doi:10.1016/s0889-8588(18)30433-7 · Cited by 82 · see citing works

In Vivo Studies of Sickle Red Blood Cells

Kaul, D. K., & Fabry, M. E. (2004) · Microcirculation · doi:10.1080/10739680490278457 · Cited by 82 · see citing works

Disorders of red cell volume regulation

Gallagher, P. G. (2013) · Current Opinion in Hematology · doi:10.1097/moh.0b013e32835f6870 · Cited by 80 · see citing works

The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

Lande, W. M., Andrews, D., Clark, M., Braham, N., Black, D., Embury, S., & Mentzer, W. C. (1988) · Blood · doi:10.1182/blood.v72.6.2056.2056 · Cited by 78 · see citing works

Treatment with NS3623, a novel Cl-conductance blocker, ameliorates erythrocyte dehydration in transgenic SAD mice: a possible new therapeutic approach for sickle cell disease

Bennekou, P., Franceschi, L. D., Pedersen, O., Lian, L., Asakura, T., Evans, G., Brugnara, C., & Christophersen, P. (2001) · Blood · doi:10.1182/blood.v97.5.1451 · Cited by 78 · see citing works

Acid pH induces formation of dense cells in sickle erythrocytes

Brugnara, C., Ha, T. V., & Tosteson, D. C. (1989) · Blood · doi:10.1182/blood.v74.1.487.487 · Cited by 78 · see citing works

Effects of alpha-thalassemia and sickle polymerization tendency on the urine-concentrating defect of individuals with sickle cell trait.

Gupta, A., Kirchner, K. A., Nicholson, R., Adams, J. G., Schechter, A. N., Noguchi, C., & Steinberg, M. H. (1991) · Journal of Clinical Investigation · doi:10.1172/jci115521 · Cited by 77 · see citing works

PATHOPHYSIOLOGY OF VASO-OCCLUSION

Ballas, S. K., & Mohandas, N. (1996) · Hematology/Oncology Clinics of North America · doi:10.1016/s0889-8588(05)70396-8 · Cited by 76 · see citing works

Sickle Cell Anemia and Major Organ Failure

Powars, D. R. (1990) · Hemoglobin · doi:10.3109/03630269009046967 · Cited by 75 · see citing works

Simultaneous polymerization and adhesion under hypoxia in sickle cell disease

Papageorgiou, D. P., Abidi, S. Z., Chang, H.-Y., Li, X., Kato, G. J., Karniadakis, G. E., Suresh, S., & Dao, M. (2018) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1807405115 · Cited by 75 · see citing works

Sickle Cell Disease: From Membrane Pathophysiology to Novel Therapies for Prevention of Erythrocyte Dehydration

Brugnara, C. (2003) · Journal of Pediatric Hematology/Oncology · doi:10.1097/00043426-200312000-00004 · Cited by 75 · see citing works

Revisiting supersaturation as a factor determining amyloid fibrillation

So, M., Hall, D. R., & Goto, Y. (2016) · Current Opinion in Structural Biology · doi:10.1016/j.sbi.2015.11.009 · Cited by 73 · see citing works

Modulation of erythrocyte potassium chloride cotransport, potassium content, and density by dietary magnesium intake in transgenic SAD mouse

Franceschi, L. D., Beuzard, Y., Jouault, H., & Brugnara, C. (1996) · Blood · doi:10.1182/blood.v88.7.2738.bloodjournal8872738 · Cited by 74 · see citing works

Anaesthesia for peculiar cells'a century of sickle cell disease

Firth, P. G. (2005) · British Journal of Anaesthesia · doi:10.1093/bja/aei129 · Cited by 72 · see citing works

3 Sickle cell disease pathophysiology

Noguchi, C. T., Schechter, A. N., & Rodgers, G. P. (1993) · Baillière s Clinical Haematology · doi:10.1016/s0950-3536(05)80066-6 · Cited by 71 · see citing works

Novel Inhibitors of the Gardos Channel for the Treatment of Sickle Cell Disease

McNaughton-Smith, G. A., Burns, J. F., Stocker, J. W., Rigdon, G. C., Creech, C., Arrington, S., Shelton, T. G., & Franceschi, L. D. (2008) · Journal of Medicinal Chemistry · doi:10.1021/jm070663s · Cited by 69 · see citing works

Potential of Three Ethnomedicinal Plants as Antisickling Agents

Nurain, I. O., Bewaji, C. O., Johnson, J. S., Davenport, R. D., & Zhang, Y. (2016) · Molecular Pharmaceutics · doi:10.1021/acs.molpharmaceut.6b00767 · Cited by 71 · see citing works

Supersaturation-limited and Unlimited Phase Transitions Compete to Produce the Pathway Complexity in Amyloid Fibrillation

Adachi, M., So, M., Sakurai, K., Kardos, J., & Goto, Y. (2015) · Journal of Biological Chemistry · doi:10.1074/jbc.m115.648139 · Cited by 67 · see citing works

Recombinant human hemoglobins designed for gene therapy of sickle cell disease.

McCune, S. L., Reilly, M. P., Chomo, M., Asakura, T., & Townes, T. M. (1994) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.91.21.9852 · Cited by 65 · see citing works

The initiation and progression of sickle cell nephropathy

Wesson, D. E. (2002) · Kidney International · doi:10.1046/j.1523-1755.2002.00363.x · Cited by 65 · see citing works

Clinical management of adult sickle-cell disease

Bartolucci, P., & Galactéros, F. (2012) · Current Opinion in Hematology · doi:10.1097/moh.0b013e328351c35f · Cited by 64 · see citing works

Sickling of red blood cells through rapid oxygen exchange in microfluidic drops

Abbyad, P., Tharaux, P., Martin, J. L., Baroud, C. N., & Alexandrou, A. (2010) · Lab on a Chip · doi:10.1039/c004390g · Cited by 61 · see citing works

The Structure of the Cataract-Causing P23T Mutant of Human γD-Crystallin Exhibits Distinctive Local Conformational and Dynamic Changes,

Jung, J., Byeon, I. L., Wang, Y., King, J. W., & Gronenborn, A. M. (2009) · Biochemistry · doi:10.1021/bi802292q · Cited by 62 · see citing works

Sickle cell states and the anaesthetist

Esseltine, D. W., Baxter, M. R. N., & Bevan, J. C. (1988) · Canadian Journal of Anesthesia/Journal canadien d anesthésie · doi:10.1007/bf03010861 · Cited by 61 · see citing works

Pathophysiological-Based Approaches to Treatment of Sickle Cell Disease

Steinberg, M. H., & Brugnara, C. (2003) · Annual Review of Medicine · doi:10.1146/annurev.med.54.101601.152439 · Cited by 61 · see citing works

Sickle cell vasoocclusion: Many issues and some answers

Kaul, D. K., & Nagel, R. L. (1993) · Cellular and Molecular Life Sciences · doi:10.1007/bf01928783 · Cited by 60 · see citing works

Extracellular fluid tonicity impacts sickle red blood cell deformability and adhesion

Carden, M. A., Fay, M. E., Lu, X., Mannino, R. G., Sakurai, Y., Ciciliano, J. C., Hansen, C. E., Chonat, S., Joiner, C. H., Wood, D. K., et al. (2017) · Blood · doi:10.1182/blood-2017-04-780635 · Cited by 60 · see citing works

A FISIOPATOLOGIA DA ANEMIA FALCIFORME

Manfredini, V., de Castro, S. M., Wagner, S. C., & Benfato, M. D. S. (2013) · Infarma - Ciências Farmacêuticas · Cited by 58 · see citing works

Haptoglobin, alpha‐thalassaemia and glucose‐6‐phosphate dehydrogenase polymorphisms and risk of abnormal transcranial Doppler among patients with sickle cell anaemia in Tanzania

Cox, S. E., Makani, J., Soka, D., L’Esperence, V., Kija, E., Domínguez-Salas, P., Newton, C. R. J. C., Birch, A. A., Prentice, A. M., & Kirkham, F. J. (2014) · British Journal of Haematology · doi:10.1111/bjh.12791 · Cited by 57 · see citing works

Diminished cerebral oxygen extraction and metabolic rate in sickle cell disease using T2 relaxation under spin tagging MRI

Bush, A., Coates, T. D., & Wood, J. C. (2017) · Magnetic Resonance in Medicine · doi:10.1002/mrm.27015 · Cited by 56 · see citing works

Membrane Transport in Sickle Cell Disease

Gibson, J. S., & Ellory, J. C. (2002) · Blood Cells Molecules and Diseases · doi:10.1006/bcmd.2002.0515 · Cited by 55 · see citing works

Effect of other hemoglobins on gelation of sickle cell hemoglobin.

Sunshine, H. R. (1982) · PubMed · Cited by 3 · see citing works

Solidity of sickle hemoglobin gels: relevance to pathophysiology of sickling disorders

Danish, E. H., Harris, J. W., & Oh, K. (1989) · Cleveland Clinic Journal of Medicine · doi:10.3949/ccjm.56.8.793 · Cited by 1 · see citing works

Hemoglobin gelation.

Adachi, K., & Asakura, T. (1980) · PubMed · Cited by 1 · see citing works

The physical chemistry of the gelation of sickle hemoglobin.

Rw, B. (1982) · PubMed · Cited by 2 · see citing works

[The hemoglobins of sickle-cell blood].

Roche, J., Derrien, Y., Gallais, P., & Roques, M. (1952) · PubMed · Cited by 1 · see citing works