Membrane transport in sickle cell disease.
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.
D000755:Anemia, Sickle Cell / Q000097:blood* / Q000209:etiology / Q000378:metabolism; D000818:Animals; D001692:Biological Transport; D002463:Cell Membrane Permeability; D004910:Erythrocyte Membrane / Q000378:metabolism* / Q000473:pathology; D004913:Erythrocytes, Abnormal / Q000378:metabolism / Q000648:ultrastructure; D006801:Humans; D026901:Membrane Transport Proteins / Q000378:metabolism / Q000502:physiology; D010100:Oxygen / Q000502:physiology
D026901:Membrane Transport Proteins; D010100:Oxygen