Red Blood Cell Adhesion in Sickle Cell Disease

biophysicalsociety

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Millions worldwide live with sickle cell disease, the most common inherited blood disorder. Sickle cell disease is due to a single-point mutation in the ÿ-globin gene resulting in the production of abnormal hemoglobin. In the deoxygenated state, hemoglobin polymerizes to form relatively stiff filaments forcing red blood cells to assume an irregular shape. It is these “sickled” red blood cells that are thought to significantly contribute to, if not initiate, occlusion of small blood vessels resulting in microvascular infarction, severe pain, widespread organ dysfunction, and early mortality.  The hallmark of the disease is the development of spontaneous, intermittent, disabling episodes of severe pain called vaso-occlusive episodes.

Our article discusses adhesion of normal and sickle cell disease human erythrocytes to endothelial laminin.  Erythrocyte adhesion to endothelium is thought to be a critical mediator of the complicated process of vaso-occlusion in sickle cell disease. This translational work is a collaboration between investigators…

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