Reaping of nitric oxide by sickle cell disease

摘要

Sickle cell disease (SCD) is the most common genetic disease among Afri- can Americans, with an 8/100 incidence of the trait among this population. This au- tosomal recessive disorder involves a sin- gle amino acid substitution in the beta subunit of hemoglobin, forming an abnor- mal protein (hemoglobin S) that after deoxygenation results in polymerization and the consequent characteristic sickle- like shape of the erythrocytes. The patho- genesis of this disease has classically been attributed to the effects of passage of the rigid malformed cells through the vascu- lature resulting in abnormal blood flow caused by physical trapping or increased adhesion of the sickled cell to the micro- vascular endothelium, commonly referred to as vaso-occlusive crisis.