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A Model of the Molecular Aggregate Processes of Hemoglobin S. Absence of Cristallization

  • Autores: Carlos Cabal Mirabal, Iván Ruiz Chaveco
  • Localización: Integración: Temas de matemáticas, ISSN 0120-419X, Vol. 26, Nº. 1, 2008 (Ejemplar dedicado a: Revista Integración), págs. 13-22
  • Idioma: inglés
  • Títulos paralelos:
    • A Model of the Molecular Aggregate Processes of Hemoglobin S. Absence of Cristallization
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  • Resumen
    • español

      Los mecanismos de formación molecular agregada desempeña un papel importante en la interpretación de la patofisiología de la anemia de células falciformes (o anemia drepanocítica), y en la selección de las estrategias terapéuticas a seguir.  Aquí se propone un mecanismo y un modelo matemático. el modelo postula la existencia de microtúbulos defectivos formados por deoxihemoglobina S y oxihemoglobina S, y explica la dependencia de la polimerización de la concentración de hemoglobina, la temperatura y la presión parcial de oxígeno. El análisis hace énfasis en la polimerización de hemoglobina S en la ausencia de cristalización. La acción de otras clases de hemoglobinas en la formación molecular agregada puede ser explicada.

    • English

      The molecular aggregate formation mechanisms play a major role in the interpretation of the pathophysiology of Sickle Cell disease and in the selection of the therapeutic strategies to follow.  A mechanism and a mathematical model are proposed. The model postu-lates the existence of defective microtubules formed by deoxy hemoglobin S and oxy hemoglobin S, and explains the dependence of polymerization on hemoglobin concentration, temperature, and partial oxygen pressure. The analysis focuses on the polymerization of hemoglobin S in the absence of crystallization. The action of other kinds of hemoglobin in the molecular aggregate formation process can be explained.   

  • Referencias bibliográficas
    • Citas [1] G.R. Serjeant, Sickle Cell Disease, Oxford University Press, UK, 1994.
    • [2] W.A. Eaton, J. Hofrichther, “Sickle Cell Hemoglobin Polymerization”, Advances in Protein Chemistry. 40 (1990) 63-279.
    • [3] C.T. Noguchi, A.N. Schechter, “The Intracellular Polymerization of Sickle Hemoglobin and its Relevance to Sickle Cell Disease”, Blood....
    • [4] G.M. Brittenham, A.N. Schechter, C.T. Noguchi, “Hemoglobin S Polymerization: Primary Determinant of the Hemolytic and Clinical Severity...
    • [5] J. Dean, A.N. Schechter, “Sickle-cell anemia. Molecular and cellular bases of therapeutic approaches”, N. Engl. J. Med. 299 (1978) 725-763.
    • [6] R.W. Brieehl, “Gelation of Sickle Cell Hemoglobin. IV. Phase Transition in Hemoglobin S Gels: Separate Measures of Aggregation and Solution-Gel...
    • [7] J. Han, J. Herzfeld, “Interpretation of the Osmotic Behavior of Sickle Cell Hemoglobin Solutions: Different Interactions Among Monomers...
    • [8] H. Chang, R.L. Nagel, “Measurement of Sickling by Controlled Temperature Increase”, Blood. 52 (1978) 1189-1195.
    • [9] R.C. Cheetham, E.R. Huehns, M.A. Rosemeyer, “Participation of Hemoglobins A, F, A2 and C in Polymerization of Hemoglobin S”, J. Mol. Biol....
    • [10] R.E. Benesch, R. Benesch, R. Edalji, S. Kwong, “Intramolecular Effects in the Polymerization of Hemoglobin S”, Biochem. Biophys. Res....
    • [11] H.R. Sunshine, J. Hofrichter, W.A. Eaton, “Gelation of Sickle Cell Hemoglobin in Mixtures with Normal Adult and Fetal Hemoglobins”, J....
    • [12] A.N. Schechter, G.P. Rodgers, “Sickle Cell Anemia, basic research in the clinic”, N. Eng. J. Med. 332 (1995) 1372-1374.
    • [13] H.F. Bunn, “Pathogenesis and Treatment of Sickle Cell Disease”, N. Engl. J. Med. 337 (1997) 762-769.
    • [14] K. Bailey, J.S. Morris, P. Thomas, G.R. Serjeant, “Fetal hemoglobin and earlymanifestation of homozygous sickle cell disease”, Arch....
    • [15] T.E. Wellems, R. Josephs, “Crystallization of Deoxyhemoglobin S by Fiber Alignment and Fusion”, J. Mol. Biol. 135(1979) 651-674.
    • [16] X.Q. Mu, B. Fairchild, “Computer models of a new deoxy-sickle cell hemoglobin fiber based on x-ray diffraction data”, Biophys. J. 61(1992)...
    • [17] T.H. Maugh, “A New Understanding of Sickle Cell Emerges. X-ray, kinetic studies paint a comprehensive picture of Sickle Cell Disease...
    • [18] A.N. Schechter, C.T. Noguchi, “Sickle Hemoglobin Polymer: Structure-Function Correlates”, Chap. 4. In Sickle Cell Disease: Basic Principles...
    • [19] A.A. Kaperonis, D.A. Handley, S. Chien, “Fibers, Crystals, and other Forms of HbS Polymers in Deoxygenated Sickle Erythrocytes”, Am....
    • [20] G.W. Dykes, R.H. Crespeau, S. J. Edelstein, “Three–dimensional reconstruction of 14-filament fibers of hemoglobin”, J. Mol. Biol. 130...
    • [21] A. Roufberg, F.A. Ferrone, “A model for the sickle hemoglobin fiber using both mutation sites”, Protein Sci. 9 (2000) 1031-1034.
    • [22] A. McPherson, “Current approaches to macromolecular crystallization”, Eur. J. Biochem. 189 (1990) 1-23.
    • [23] G. Agarwal, J.C. Wang, S. Kwong , S.M. Cohen, F.A. Ferrone, R. Josephs, R.W. Briehl, “Sickle hemoglobin Fibers: Mechanisms of Depolymerization”,...
    • [24] P.C. Weber, “Protein Crystallization”, Advan. in Protein Chemistry. 41 (1991) 1-5.
    • [25] F.A. Ferrone, J. Hofrichter, W.A. Eaton, “Kinetics of sickle hemoglobin polymerization I. Studies using temperature jump and laser photolysis...
    • [26] F.A. Ferrone, J. Hofrichter, and W.A. Eaton, “Kinetics of Sickle Hemoglobin Polymerization. II A double nucleation mechanism”, J. Mol....
    • [27] M. Ivanova, R. Jasuja, S. Kwong, R.W. Briehl, F.A. Ferrone, “Nonideality and the nucleation of sickle hemoglobin”, Biophys. J. 79 (2000)...
    • [28] V.B Makhijani., G.R. Coketet, A. Clark, “Dymanics of oxygen unloading from sickle erythrocytes”, Biophys. J. 58 (1990) 1025-1050.
    • [29] F.A. Ferrone, M. Ivanova, R. Jasuja, “Heterogeneous Nucleation Crowding in Sickle Hemoglobin. An Analytic Approach”, Biophys. J. 82 (2002)...
    • [30] M. Ivanova, R. Jasuja, S. Kwong, R.W Briehl, F.A. Ferrone, “Nonideality and the nucleation of Sickle Hemoglobin”, Biophys. J. 79 (2000)...
    • [31] C. Cabal, A. Fernández, M. Lores, E. Álvarez, J. Losada, C. Soler, E. Pérez, “Magnetic Relaxation in the Kinetics of the Polymerization...

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