Oxygen Binding and the Gelation of Sickle Cell Hemoglobin

Hofrichter, James; Sunshine, Helen R.; Ferrone, Frank A.; Eaton, William A.

doi:10.1016/b978-0-444-00631-8.50025-8

Cited by 2 publications

(2 citation statements)

References 26 publications

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“…[6] Experiments by Hofrichter (22) on mixtures of CO-hemoglobin S and unliganded hemoglobin S have shown that the data for the incorporation of partially liganded hemoglobin S molecules are best fitted with e2 = 0.2. However, Hofrichter et al (31) have recently shown that the behavior of oxygen is similar to that of CO, but with e2 = 0.4. We have used these values to compute the other two curves shown in Fig.…”

Section: Resultsmentioning

confidence: 99%

Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

Noguchi¹,

Torchia²,

Schechter³

1980

Proc. Natl. Acad. Sci. U.S.A.

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Section: Resultsmentioning

confidence: 99%

Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

Noguchi¹,

Torchia²,

Schechter³

1980

Proc. Natl. Acad. Sci. U.S.A.

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“…In addition to cell suspension studies, viscometry is also useful for determining the rheological properties of Hb S solutions (Briehl, 1981;Chien et al, 1982;Danish et al, 1987;Drasler et al, 1989). Recent experimental studies have shown that the overall rheological properties of sickle erythrocytes are strongly influenced by both cell membrane viscoelasticity (Nash et al, 1984;Evans et al, 1984;Drasler et al, 1989;Messmann et al, 1990;Hebbel, 1991) and interior Hb S gelation (Briehl, 1981;Hofrichter et al, 1981;Gabriel et al, 1981;Noguchi and Schechter, 1985;Hofrichter, 1987, 1990;Danish et al, 1987Danish et al, , 1989Clark, 1989).…”

Section: Experimental and Theoretical Backgroundmentioning

confidence: 99%

Influence of sickle hemoglobin polymerization and membrane properties on deformability of sickle erythrocytes in the microcirculation

Dong

Chadwick

Schechter

1992

Biophysical Journal

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The rheological properties of normal erythrocytes appear to be largely determined by those of the red cell membrane. In sickle cell disease, the intracellular polymerization of sickle hemoglobin upon deoxygenation leads to a marked increase in intracellular viscosity and elastic stiffness as well as having indirect effects on the cell membrane. To estimate the components of abnormal cell rheology due to the polymerization process and that due to the membrane abnormalities, we have developed a simple mathematical model of whole cell deformability in narrow vessels. This model uses hydrodynamic lubrication theory to describe the pulsatile flow in the gap between a cell and the vessel wall. The interior of the cell is modeled as a Voigt viscoelastic solid with parameters for the viscous and elastic moduli, while the membrane is assigned an elastic shear modulus. In response to an oscillatory fluid shear stress, the cell--modeled as a cylinder of constant volume and surface area--undergoes a conical deformation which may be calculated. We use published values of normal and sickle cell membrane elastic modulus and of sickle hemoglobin viscous and elastic moduli as a function of oxygen saturation, to estimate normalized tip displacement, d/ho, and relative hydrodynamic resistance, Rr, as a function of polymer fraction of hemoglobin for sickle erythrocytes. These results show the transition from membrane to internal polymer dominance of deformability as oxygen saturation is lowered. More detailed experimental data, including those at other oscillatory frequencies and for cells with higher concentrations of hemoglobin S, are needed to apply fully this approach to understanding the deformability of sickle erythrocytes in the microcirculation. The model should be useful for reconciling the vast and disparate sets of data available on the abnormal properties of sickle cell hemoglobin and sickle erythrocyte membranes, the two main factors that lead to pathology in patients with this disease.

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Oxygen Binding and the Gelation of Sickle Cell Hemoglobin

Cited by 2 publications

References 26 publications

Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

Influence of sickle hemoglobin polymerization and membrane properties on deformability of sickle erythrocytes in the microcirculation

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