Red Cell Membrane and Transport Systems: Hereditary Disorders

Ronald L Nagel, Albert Einstein College of Medicine, New York, New York, USA

Published online: April 2006

DOI: 10.1038/npg.els.0002281

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., 2003) 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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    Tse WT and Lux SE (1999) Red blood cell membrane disorders. British Journal of Haematology 104: 2–13.

Related Sub-Topics:

Cell Membranes | Membrane Proteins | Lipids and Membrane Structure | Specific Genetic Disorders | Mutational Mechanisms of Genetic Disease
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Article Title: Red Cell Membrane and Transport Systems: Hereditary Disorders
 
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]