Among the various potential antisickling agents tested, hydroxyurea (HU) has been the most
effective compound used for the treatment of patients with sickle cell disease (SCD) …

The primary pathogenic event of sickle cell anemia is the polymerization of the mutant
hemoglobin (Hb) S within the red blood cells, occurring when HbS is in deoxy state in the …

Pathology in sickle cell disease begins with nucleation-dependent polymerization of
deoxyhemoglobin S into stiff, rodlike fibers that deform and rigidify red cells. We have …

The ever-increasing body of information regarding the molecular pathogenesis of sickle cell
disease has raised expectations that a specific and effective therapy could be devised to …

Much progress has been made during the past several decades in gaining understanding
about the natural history of sickle cell disease and management approaches aimed at …

Determination of the optimal buffer for rheological studies of sickle cells is of particular
importance for in vitro screening of potential anti-sickling compounds and for ex vivo studies …

To develop an animal model for sickle cell anemia, we have created transgenic mice that
express a severe naturally occurring human sickling hemoglobin, Hb S Antilles. Due to its …

Background: Sickle cell disease causes significant morbidity and mortality and affects the
economic and healthcare status of many countries. Yet historically, the disease has not had …

Sickle cell anemia (SCA) was first described in the Western literature more than 100 years
ago. Elucidation of its molecular basis prompted numerous biochemical and genetic studies …

In an effort to study the rheologic effects of small amounts of hemoglobin S (HbS) polymer in
sickle red cells, we have used the ektacytometer, a laser diffraction couette viscometer, to …