We examined the extent to which the intracellular polymerization of sickle hemoglobin (HbS)
can account for the severity of anemia and of vaso-occlusive manifestations in the various …

Recent work has enabled us to quantitate the four variables (2, 3-DPG concentration, pHi,
non-S hemoglobin composition, and O2 saturation) that modulate the equilibrium solubility …

ICKLE CELL ANEMIA, a disease having both he-S molytic and vasocclusive components,
has been subjected to intense scrutiny at the clinical, cellular, biochemical, and molecular …

Sickle cell anaemia is associated with a mutant haemoglobin, HbS, which forms polymers in
the red blood cells of patients. The primary role of the HbS polymerization for the …

To determine the extent to which the broad distribution in intracellular hemoglobin
concentrations found in sickle erythrocytes affects the extent of intracellular polymerization of …

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

The clinical severity of various sickle hemoglobinopathies was directly related to the rate of
increase of viscosity of blood during deoxygenation. The principal determinants of the rate …

Abstract 70 years ago, Linus Pauling, the legendary genius of 20th century chemistry,
published his famous work on the molecular cause of sickle cell disease, a paper that gave …

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 …

Intracellular hemoglobin S (HbS) polymerization is most likely to be the primary determinant
of the clinical and biologic manifestations of sickle cell disease (SCD). Fetal hemoglobin …