Membrane Transport in Sickle Cell Disease

• Published in: Blood cells, molecules, & diseases Vol. 28; no. 3; pp. 303 - 314
• Main Authors: Gibson, J.S., Ellory, J.C.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-05-2002
• Subjects: Anemia, Sickle Cell - blood; Anemia, Sickle Cell - etiology; Anemia, Sickle Cell - metabolism; Animals; Biological Transport; Cell Membrane Permeability; Erythrocyte Membrane - metabolism; Erythrocyte Membrane - pathology; Erythrocytes, Abnormal - metabolism; Erythrocytes, Abnormal - ultrastructure; Humans; Membrane Transport Proteins - metabolism; Membrane Transport Proteins - physiology; Oxygen - physiology
• ISSN: 1079-9796; 1096-0961
• DOI: 10.1006/bcmd.2002.0515

ABSTRACT
We have reviewed here a number of membrane transport events in red cells from normal individuals and sickle cell patients which respond to changes in O
2 tension. Some deoxygenation-induced changes in membrane permeability are unique to HbS cells and contribute to their dehydration and subsequent sickling. Polymerization of HbS, or specific oxidant damage (or altered redox potential), is a likely factor underlying the abnormal behavior. The key regulatory sites within the membrane or associated proteins remain uncertain and their identity will form the focus of future research. A model for sickle cell dehydration is presented. Inhibition of these permeability changes represents possible avenues for future chemotherapy to ameliorate the condition.