Red cell membrane sialoglycoptein β in homozygous and heterozygous 4.1(−) hereditary elliptocytosis

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Abstract

Sialoglycoprotein β, a minor sialoglycoprotein of the red cell membrane, was studied in homozygous and heterozygous 4.1 (−) hereditary elliptocytosis, a variety of hereditary elliptocytosis characterized by total or partial absence of protein 4.1. Erythrocytes were treated with the periodic acid-NaB3H4 procedure. Following polyacrylamide gel electrophoresis in the presence of SDS, labelled sialoglycoproteins were revealed by fluorography. (i) In the ghosts from the 4.1(−) homozygote, sialoglycoprotein β was sharply decreased. It is not sure whether the residual material is sialoglycoprotein β itself, or a distinct sialoglycoprotein migrating in the same place. In long exposure fluorograms, sialoglycoprotein γ (a sialoglycoprotein related to sialoglycoprotein β) also turned out to be reduced. In the homozygote's Triton-shells, sialoglycoproteins β and γ appeared completely absent. (ii) In the 4.1(−) heterozygote, sialoglycoprotein β appeared slightly reduced, whereas sialoglycoprotein γ appeared normal. Both of these proteins were extracted in seemingly normal amounts in the Triton-shells. These observations bring further support to the view that there is an inteaction between skeletal membrane protein 4.1 and sialoglycoprotein β, that is additional to other interactions between the former protein and the lipid bilayer and/or other transmembrane proteins.

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