Current diagnostic approach and screening methods for hereditary spherocytosis

• Main Author: Paola Bianchi
• Format: Article
• Language: English
• Published: PAGEPress Publications 2013-03-01
• Series: Thalassemia Reports
• Online Access: http://www.pagepressjournals.org/index.php/thal/article/view/1500

The plasma membrane of the erythrocyte accounts for all of its antigenic, transport, and mechanical characteristics, particularly its ability to undergo large passive deformations during repeated passage through the narrow capillaries of the microvasculature, throughout its 120-day life span. The determinant of normal membrane cohesion is the system of vertical linkages between phospholipid bilayer and membrane skeleton, formed by the interactions of the cytoplasmic domains of various membrane proteins with the spectrin-based skeletal network. Band 3 and RhAG provide such links by interacting with ankyrin, which in turn binds to b-spectrin. Protein 4.2 binds to both band 3 and ankyrin and can regulate the avidity of the interaction between band 3 and ankyrin. Glycophorin C, band 3, XK, Rh, and Duffy all bind to protein 4.1R, the third member of the ternary junctional complex with bspectrin and actin. Horizontal linkages in the membrane skeleton due to spectrin dimer-dimer interaction or spectrin-actin-protein 4.1 junctional complex, confer to the erythrocytes elasticity and deformability...