Erythrocyte Membrane Disorders

Abstract

Erythrocyte membrane disorders, such as hereditary spherocytosis and elliptocytosis, usually present with abnormal red cell shapes, hemolysis and anemia. Recent genomic research has shown that defects in proteins that constitute the erythrocyte membrane skeleton are often the causes of these disorders.

Keywords: spherocytosis; elliptocytosis; pyropoikilocytosis; spectrin; ankyrin

Figure 1.

Structure of the erythrocyte membrane. A membrane skeleton lies on the inner surface of the lipid bilayer of the erythrocyte membrane and is important in maintaining the structural integrity of red blood cells. The membrane skeleton comprises a hexagonal network of α‐ and β‐spectrin peptides intertwined to form a strong but flexible scaffold. The peripheral membrane proteins ankyrin, protein 4.2, actin and protein 4.1 anchor the spectrin scaffold to the lipid bilayer by attaching to the major erythrocyte integral membrane proteins, band 3 and the glycophorins.

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References

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Further Reading

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Tse, Willian T(Jan 2006) Erythrocyte Membrane Disorders. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1038/npg.els.0006094]