The development of new methods for manipulating the mouse genome, including transgenic
and embryonic stem (ES) cell knockout technology, combined with greatly improved genetic …

Vasocclusive events in the sickle-cell syndromes have multiple determinants: first and
foremost is the capacity of red cells to undergo intracellular polymerization of deoxy HbS …

Intravascular hemolysis describes the relocalization of heme and hemoglobin (Hb) from
erythrocytes to plasma. We investigated the concept that erythrocyte membrane …

Sickle cell disease (SCD) is caused by a single point mutation in the human βA globin gene
that results in the formation of an abnormal hemoglobin [HbS (α2βS 2)]. We designed a βA …

Sickle human hemoglobin (Hb) confers a survival advantage to individuals living in endemic
areas of malaria, the disease caused by Plasmodium infection. As demonstrated hereby …

Vascular occlusion is the major cause of morbidity and mortality in sickle cell disease but its
mechanisms are poorly understood. We demonstrate by using intravital microscopy in mice …

To create mice expressing exclusively human sickle hemoglobin (HbS), transgenic mice
expressing human α-, γ-, and βS-globin were generated and bred with knockout mice that …

When transgenic mice that expressed human sickle hemoglobin were mated with mice
having knockout mutations of the mouse α-and β-globin genes, animals were produced that …

Patients affected by β-thalassemia major require lifelong transfusions because of insufficient
or absent production of the β chain of hemoglobin (Hb). A minority of patients are cured by …

Patients with sickle cell disease suffer from painful crises associated with disseminated vaso-
occlusions, increased circulating erythrocyte microparticles (MPs), and thrombospondin-1 …