Red Cell Membrane and Transport Systems: Hereditary Disorders

Abstract

The structure and function of the red cell membrane and associated ion transporters play an important role in the pathology of red cell genetic defects.

Keywords: red cell membrane; ion transporters; hereditary disorders; genetic defects

Figure 1.

Band 3 and also glycophorin proteins (not depicted in this figure) are the most abundant proteins that transverse the bilipid red cell membrane. Recent evidence (Nicolas et al., ) suggest that the Rh complex represents a major interaction site between the membrane lipid bilayer and the red cell skeleton: ankyrin‐R is capable of interacting directly with the C‐terminal cytoplasmic domain of Rh and RhAG polypeptides. These results identify the Rh/RhAG–ankyrin complex as a new interaction site between the red cell membrane and the spectrin‐based skeleton, the disruption of which might result in the stomato‐spherocytosis typical of Rh null red cells.

Figure 2.

Diagram of transporters present in the human red cell membrane. Each transporter is defined as to the cation or anion involved in their particular transport phenomena.

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Further Reading

Cooke BM, Mohandas N and Coppel RL (2004) Malaria and the red blood cell membrane. Seminars in Hematology 41: 173–188.

Drew C, Ball V, Robinson H, Clive Ellory J and Gibson JS (2004) Oxygen sensitivity of red cell membrane transporters revisited. Bioelectrochemistry 62: 153–158.

Eda S and Sherman IW (2002) Cytoadherence of malaria‐infected red blood cells involves exposure of phosphatidylserine. Cellular Physiology and Biochemistry 12: 373–384.

Gov N, Zilman AG and Safran S (2003) Cytoskeleton confinement and tension of red blood cell membranes. Physical Review Letters 90: 228101.

Kay MM (2004) Band 3 and its alterations in health and disease. Cellular and Molecular Biology 50: 117–138.

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Thomas SL and Lew VL (2004) Plasmodium falciparum and the permeation pathway of the host red blood cell. Trends in Parasitology 20: 122–125.

Tse WT and Lux SE (1999) Red blood cell membrane disorders. British Journal of Haematology 104: 2–13.

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How to Cite close
Nagel, Ronald L(Apr 2006) Red Cell Membrane and Transport Systems: Hereditary Disorders. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1038/npg.els.0002281]