Sickle cell disease (SCD) is described as the first identi-fied “molecular” disease since its
manifestations stem from a substitution of valine for glutamic acid in the structure of the β …

To understand the physical basis of the wide variety of shapes of deoxygenated red cells
from patients with sickle cell anemia, we have measured the formation rate and volume …

Sickle cell disease, caused by a mutation in the β-hemoglobin gene, is a Mendelian disorder
with a very diverse phenotype. The primary cause of disease pathophysiology is the …

Background: Sickle cell disease (SCD) is caused by polymerization of Hemoglobin S,
resulting in red blood cell (RBC) sickling, RBC destruction, vaso-occlusive episodes and …

Although homozygous sickle cell disease is often clinically severe, the corresponding
heterozygous state, sickle cell trait, is almost completely benign despite the fact that there is …

Polymerization of HbS and cell sickling are the prime pathophysiological events in sickle cell
disease (SCD). Over the last 30 years, a substantial understanding at the molecular level …

The complex, frequently devastating, multi-organ pathophysiology of sickle cell disease has
a single root cause: polymerization of deoxygenated sickle hemoglobin. A logical approach …

Five years experience using 5AZA and HU in a limited number of patients with SS disease,
have resulted in the following conclusions. First, these drugs can rapidly induce increases in …

Sickle cell disease is a systemic disorder that is caused by a mutation (Glu6Val) in the gene
that encodes β globin. The sickle hemoglobin molecule (HbS) is a tetramer of two α-globin …

We analyzed the effects of protein and water nonideality and of erythrocyte heterogeneity on
the polymerization of hemoglobin S in cells where there were significant amounts of non-S …