All the following protein defects can cause hereditary spherocytosis EXCEPT
Hereditary spherocytosis (HS) is an inherited disorder caused by intrinsic defects in the red cell membrane skeleton that render red cells spheroid, less deformable, and vulnerable to splenic sequestration and destruction.Various mutations involving a-spectrin, b-spectrin, ankyrin, band 4.2, or band 3 that weaken the interactions between these proteins causered cells to lose membrane fragments. To accommodate the resultant change in the ratio of surface area to volume these cells adopt a spherical shape.Spherocytic cells are less deformable than normal ones and therefore become trapped in the splenic cords, where they are phagocytosed by macrophages.Reference: Robbins and Cotran Pathologic Basis of Disease; 9th edition; Chapter 14; Red Blood Cell and Bleeding Disorders
Ankyrin
alfa-Spectrin
Glycophorin C
beta spectrin
General pathology