Kinetics of sickle hemoglobin polymerization: III. Nucleation rates determined from stochastic fluctuations in polymerization progress curves

J Hofrichter - Journal of molecular biology, 1986 - Elsevier
The polymerization kinetics of sickle cell hemoglobin are found to exhibit stochastic
variations when observed in very small volumes (~ 10− 10 cm 3). The distribution of …

[引用][C] Hemoglobin S gelation and sickle cell disease

WA Eaton, J Hofrichter - 1987

Oxygen binding by sickle cell hemoglobin polymers

HR Sunshine, J Hofrichter, FA Ferrone… - Journal of molecular …, 1982 - Elsevier
The thermodynamics of sickle cell hemoglobin gelation in the presence of oxygen has been
investigated by measuring the fractional saturation of the solution and polymer phases, and …

Sparing effect of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S at physiologic ligand saturations.

WN Poillon, BC Kim, GP Rodgers… - Proceedings of the …, 1993 - National Acad Sciences
Recent interest in therapies for sickle cell anemia based on elevating fetal Hb has made
accurate estimates of the sparing effect of fetal Hb (Hb F) and other non-sickle Hbs on sickle …

Gelation of sickle cell hemoglobin in mixtures with normal adult and fetal hemoglobins

HR Sunshine, J Hofrichter, WA Eaton - Journal of molecular biology, 1979 - Elsevier
We report the results of thermodynamic and kinetic studies on the gelation of mixtures of
sickle cell (S) deoxyhemoglobin with normal human adult (A) and fetal (F) …

2, 3-Diphosphoglycerate and intracellular pH as interdependent determinants of the physiologic solubility of deoxyhemoglobin S

WN Poillon, BC Kim - 1990 - ashpublications.org
Abstract We have established that 2, 3-diphosphoglycerate (2, 3-DPG) content and
intracellular pH exert separate, but interdependent, effects on the equilibrium solubility (csat) …

3 Sickle cell disease pathophysiology

CT Noguchi, AN Schechter, GP Rodgers - Baillière's clinical haematology, 1993 - Elsevier
The primary pathophysiological event in the erythrocytes of individuals with the various
sickle syndromes is the intracellular aggregation or polymerization of sickle haemoglobin …

[HTML][HTML] Covalent binding of glutathione to hemoglobin. I. Inhibition of hemoglobin S polymerization.

MC Garel, C Domenget, J Caburi-Martin… - Journal of Biological …, 1986 - Elsevier
Thiol reagents react with cysteine beta 93 of hemoglobin and as a result increase the
oxygen affinity of hemoglobin. In the present studies we have used a thiol-disulfide …

Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

CT Noguchi, DA Torchia… - Proceedings of the …, 1980 - National Acad Sciences
We have used 13C/1H magnetic double-resonance spectroscopy to measure the amount of
sickle hemoglobin polymer within sickle erythrocytes as a function of oxygen saturation. We …

Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.

CT Noguchi, DA Torchia… - The Journal of clinical …, 1983 - Am Soc Clin Investig
To determine the extent to which the broad distribution in intracellular hemoglobin
concentrations found in sickle erythrocytes affects the extent of intracellular polymerization of …