Structural specificities in acylation of hemoglobin and sickle hemoglobin by diaspirins.

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...

Intraerythrocytic Hemoglobin Crystals in Sickle Cell-hemoglobin C Disease.

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...

Hemoglobin S gelation and sickle cell disease.

By William A. Eaton and James Hofrichter T HE FUNDAMENTAL cause ofsickle cell disease is the decreased deformability of the sickled red cell produced by gelation of hemoglobin S. Partial inhibition of gelation should therefore reduce clinical severity, while complete inhibition should result in a “cure.” These basic ideas have stimulated an enormous effort to understand the gelation process in ...

Fetal hemoglobin in sickle cell anemia

Fetal hemoglobin (HbF) is the major genetic modulator of the hematologic and clinical features of sickle cell disease, an effect mediated by its exclusion from the sickle hemoglobin polymer. Fetal hemoglobin genes are genetically regulated, and the level of HbF and its distribution among sickle erythrocytes is highly variable. Some patients with sickle cell disease have exceptionally high level...