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 …

Sickle cell anemia occurs in individuals who are homozygous for a single nucleotide
substitution in codon 6 of the beta-globin gene. This single mutation leads to the formation of …

The extent of intracellular polymerization of hemoglobin S, leading to loss of erythrocyte
deformability and eventual morphological sickling, is primarily determined by oxygen …

Sickle cell anemia is a genetic disorder characterized by mutant hemoglobin (Hb)
polymerization and resultant cell deformation (sickling) under conditions of reduced oxygen …

The dominant assumption central to most treatments for sickle cell anemia has been that
replacement of sickle hemoglobin (HbS) by fetal hemoglobin (HbF) would have major …

The data available clearly establish that the hyperconcentration of hemoglobin C and S
inside SC cells is the main and driving mechanism for the pathologic behavior of these cells …

The present molecular-level understanding of polymerization and sickling is reviewed for 2
central questions in sickle hemoglobin pathophysiology, viz., what determines when cells …

Sickle hemoglobin (HbS), caused by a point mutation in the β-globin gene of hemoglobin,
polymerizes when deoxygenated. The pathophysiology of sickle cell disease results from …

The beta S mutation responsible for sickle cell disease (SCD) was identified in 1949. This
mutation always consists in a T for A substitution at codon 6 of the beta-globin chain …