Abnormal degradation of erythrocyte membrane proteins in hereditary spherocytosis.

The degradation rate of erythrocyte membrane proteins by membrane-bound proteases was compared in healthy controls and in patients with hereditary spherocytosis (HS). An increased degradation rate of spectrin and a decreased digestion rate of Band 3 were found in HS patients. These differences may be due to altered accessibilities of membrane proteins to proteases and/or to changes in the pattern of membrane-associated proteases and may be connected with the decrease in the spectrin: Band 3 ratio reported for erythrocyte membranes in HS.