Rapid and inefficient kinetics of sickle hemoglobin fiber growth

Sickle cell-hemoglobin C disease. Quantitative determination of iron kinetics and hemoglobin synthesis.

Kinetics of the carbamylation of the amino groups of sickle cell hemoglobin by cyanate.

The NH2-terminal valine residues of both chains of oxyhemoglobin S are carbamylated 50 to 100 times faster at pH 7.4 than the a-NH2 groups of the lysine residues of the protein up to a level of 1 carbamyl group per hemoglobin molecule. The pseudo-first order rate constants for the carbamylation of the NHz-terminal residues of carbonmonoxyhemoglobins A and S are identical for the proteins either...

Fiber depolymerization: fracture, fragments, vanishing times, and stochastics in sickle hemoglobin.

The well-characterized rates, mechanisms, and stochastics of nucleation-dependent polymerization of deoxyhemoglobin S (HbS) are important in governing whether or not vaso-occlusive sickle cell crises will occur. The less well studied kinetics of depolymerization may also be important, for example in achieving full dissolution of polymers in the lungs, in resolution of crises and/or in minimizin...

Denatured hemoglobin in sickle erythrocytes.

To study the nature of numerous inclusion bodies seen in red cells from patients with sickle cell disease (Hb SS), we have prepared red cell ghosts free of oxyhemoglobin and analyzed them by spectrophotometric and heme extraction methods. The absorption spectrum in the visible region of the ghost suspensions was typical of hemichromes. The spectrum was similar to that of denatured hemoglobin re...

Sickle hemoglobin: a specific radioimmunoassay.

For the quantitation of hemoglobin S, a radioimm unoassay has been developed which is specific and highly sensitive. Hemoglobin S was purified by column chromatography and injected with complete Freund’s adjuvant into goats. Each goat serum was tested for reactivity against hemoglobins A and S by immunodiffusion and by quantitative precipitation. Hemoglobin A reactivity was removed by absorptio...