title: Inherited sialoglycoprotein deficiencies in human erythrocytes of type En[a-].
abstract: We have investigated the membranes of erythrocytes from a family in which there is a genetic defect [previously described as the En[a-] condition[ resulting in the loss of the major erythrocyte sialoglycoprotein [PAS-i]. The results show that two different types of sialoglycoprotein deficiency can be distinguished within this family. We suggest that the En[a-] group of variants is more appropriately described as a class of sialoglycoprotein deficient erythrocytes. Using a new technique it is shown that the blood group M antigen of normal erythrocytes is found only on the erythrocyte sialoglycoprotein while in this family the M antigen is found on membrane components other than the sialoglycoprotein. Our results suggest that the amino acid sequence of the sialoglycoprotein is important in defining the difference between the blood group M and N antigens in normal erythrocytes.
mesh_terms: D010131:Aminosalicylic Acid / Q000378:metabolism; D001789:Blood Group Antigens; D004591:Electrophoresis, Polyacrylamide Gel; D004910:Erythrocyte Membrane / Q000032:analysis* / Q000378:metabolism; D004912:Erythrocytes / Q000032:analysis*; D005260:Female; D030342:Genetic Diseases, Inborn; D006021:Glycophorins / Q000172:deficiency*; D006801:Humans; D037102:Lectins / Q000494:pharmacology; D008951:MNSs Blood-Group System; D008297:Male; D008565:Membrane Proteins / Q000032:analysis; D010375:Pedigree; D012795:Sialoglycoproteins / Q000172:deficiency*
chemical_list: D001789:Blood Group Antigens; D006021:Glycophorins; D037102:Lectins; D008951:MNSs Blood-Group System; D008565:Membrane Proteins; D012795:Sialoglycoproteins; D010131:Aminosalicylic Acid