Deficient Red-Cell Spectrin in Severe, Recessively Inherited Spherocytosis

Abstract

RED-CELL-membrane architecture has been extensively studied in recent years.1 , 2 When the cells are hypotonically lysed and washed free of hemoglobin, the resulting membrane ghosts consist of the lipid bilayer and associated proteins. When ghosts are extracted with non-ionic detergents, lipid and integral membrane proteins are removed, leaving a cytoskeleton3 that is thought to be responsible for the shape, strength, and reversible deformability of the cell. The cytoskeleton consists of an assembly of polypeptides: spectrin (the largest and most abundant protein of the membrane), band 4.1, and actin. The cytoskeleton is attached to the membrane by the association of spectrin with . . .