Article
Sickle cell disease, vasculopathy, and therapeutics.
Annual review of medicine - 1 Jan 2013
Kassim Adetola A, DeBaun Michael R
Abstract excerpt
Sickle cell disease (SCD) is caused by a mutation in both beta globin genes, resulting in chronic hemolysis and multiorgan disease that ultimately leads to premature death. Although hemoglobin S (HbS) polymerization and vaso-occlusion are central to the pathogenesis of SCD, overlapping pathways implicated in SCD-related endothelial dysfunction include hemolysis, defects in nitric oxide metabolism,...
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