Normal membrane function of abnormal beta-related erythrocyte sialoglycoproteins.
Red cells totally deficient in beta and gamma sialoglycoproteins (the Leach type of Gerbich-negative) are elliptocytic and have altered membrane physical properties as evidenced by marked decreases in both membrane mechanical stability and membrane deformability. Red cells from individuals who are of the Gerbich and Yus phenotypes of Gerbich-negative are also deficient in beta and gamma sialoglycoproteins, but possess abnormal beta-related sialoglycoproteins. In order to determine if these beta-related sialoglycoproteins can functionally substitute for normal sialoglycoproteins, we measured membrane deformability and stability of red cells of the Gerbich and Yus phenotypes. In contrast to the red cells of the Leach phenotype, cells of Gerbich and Yus phenotypes were found to have normal membrane deformability and stability. Moreover, flow cytometric analysis using a monoclonal anti-beta antibody revealed that the Gerbich and Yus phenotype red cells expressed the beta-related sialoglycoprotein to the same extent as its normal counterpart on normal cells. Based on these data, we suggest that the abnormal beta-related sialoglycoproteins can functionally substitute for normal beta and gamma sialoglycoproteins.
Reid
M E
ME
Department of Laboratory Medicine, San Francisco General Hospital, CA 94110.
Anstee
D J
DJ
Jensen
R H
RH
Mohandas
N
N
D004907:Erythrocyte Deformability; D004910:Erythrocyte Membrane / Q000502:physiology; D004913:Erythrocytes, Abnormal / Q000032:analysis / Q000502:physiology*; D006021:Glycophorins / Q000032:analysis / Q000502:physiology*; D006801:Humans; D012795:Sialoglycoproteins / Q000502:physiology*
D006021:Glycophorins; D012795:Sialoglycoproteins