Although it has been known for more than 60 years that the cause of sickle cell disease is
polymerization of a hemoglobin mutant, hydroxyurea is the only drug approved for treatment …

Sickle cell disease (SCD) is characterized by the presence of sickle hemoglobin (HbS),
which has the unique property of polymerizing when deoxygenated. The sickling process is …

By William A. Eaton, James Hofrichter, and Philip D. Ross T HE FACTORS which determine
the frequency of crises and overall severity in sickle cell disease are still not clear. Although …

Although the root cause of sickle cell disease is the polymerization of hemoglobin S (HbS) to
form fibers that make red cells less flexible, most drugs currently being assessed in clinical …

A major driver of the pathophysiology of sickle cell disease (SCD) is polymerization of
deoxygenated haemoglobin S (HbS), which leads to sickling and destruction of red blood …

In the last few years, the understanding of the molecular basis of sickle cell disease has
progressed rapidly. It is now possible to describe the structure of the gel of polymerized …

The hallmark of sickle cell disease is the polymerization of sickle haemoglobin due to a point
mutation in the β‐globin gene (HBB). Under low oxygen saturation, sickle haemoglobin …

The primary pathophysiological event in the erythrocytes of individuals with the various
sickle syndromes is the intracellular aggregation or polymerization of sickle haemoglobin …

Evaluation of New Therapies To facilitate the application of the previously discussed assays
of gelation, sickling and rheology to new drugs, the Sickle Cell Disease Branch of the …

In sickle cell disease, the aberrant assembly of hemoglobin fibers induces changes in red
blood cell morphology and stiffness, which leads to downstream symptoms of the disease …