Dependence of the permanent deformation of red blood cell membranes on spectrin dimer-tetramer equilibrium: implication for permanent membrane deformation of irreversibly sickled cells

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Dependence of the permanent deformation of red blood cell membranes on spectrin dimer-tetramer equilibrium: implication for permanent membrane deformation of irreversibly sickled cells.

Red blood cells (RBCs) in sickle cell anemia, transformed into a sickled shape by prolonged deoxygenation, or normal RBCs deformed by a prolonged micropipette aspiration become permanently stabilized in their abnormal shape. This semisolid plastic behavior is thought to involve an irreversible reorganization of the membrane skeleton, but the exact nature of this skeletal rearrangement is not kn...

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Irreversibly sickled cells (ISC's) are circulating erythrocytes in patients with sickle cell disease that retain a sickled shape even when oxygenated. Evidence points to a membrane defect that prevents the return of these cells to the normal biconcave shape. The erythrocyte membrane protein spectrin is believed to help control erythrocyte shape and deformability. Recent studies suggest that nor...

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ژورنال

عنوان ژورنال: Blood

سال: 1993

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v81.2.522.bloodjournal812522