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

Sickle cell syndromes are a group of inherited disorders of haemoglobin structure that have
no cure in adults at the present time. Bone marrow transplantation in children has been …

Polymerization of de oxyhemoglobin S within sickle erythrocytes is of cardinal importance to
each of the clinical complications of sickle cell anemia. Sickle gene expression, however, is …

Over 70,000 people live with sickle cell disease in the United States and multitudes world
wide. About 2000 afflicted babies are born in this country each year. In African countries …

Sickle cell disease is caused by a mutation in the β-globin chain of the haemoglobin
molecule. Sickle haemoglobin, the result of this mutation, has the singular property of …

The concurrence of sickle cell anemia and alpha-thalassemia results in less severe
hemolytic anemia apparently as a result of reduced intraerythrocytic concentration of …

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 …

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

Sickle cell trait usually is not regarded as a disease state because it has complications that
are either uncommon or mild. Nevertheless, under unusual circumstances serious morbidity …