Abnormal binding of spectrin to the membrane of erythrocytes in some cases of hereditary spherocytosis.
In two cases of hereditary spherocytosis that we have examined, spectrin was bound abnormally tightly to the erythrocyte membrane, and could not be released by low ionic strength dialysis. This type of behaviour occurs in normal red cells only after heating above 50 degrees C. It appears that some cases of spherocytosis may be due to the presence of a protein which is abnormally temperature sensitive.
D004910:Erythrocyte Membrane; D004912:Erythrocytes; D006801:Humans; D008565:Membrane Proteins / Q000378:metabolism*; D011485:Protein Binding; D013049:Spectrin / Q000378:metabolism*; D013103:Spherocytosis, Hereditary / Q000097:blood*; D013696:Temperature
D008565:Membrane Proteins; D013049:Spectrin