The abnormal phosphorylation of spectrin in human hereditary spherocytosis.
The phosphorylation of the proteins of the erythrocyte membrane of patients suffering from hereditary spherocytosis is investigated in intact erythrocytes by their incubation in the presence of radioactive inorganic phosphate. Examination of the phosphorylated components by high-resolution two-dimensional gel electrophoresis reveals only one defect in the pathological membranes, a depressed phosphorylation of the smaller polypeptide of spectrin; band 2. The phosphorylation of band 2 is measured with reference to the phosphorylation of syndein (2.1 + 2.2 + 2.3). In patients showing overt clinical symptoms and for whom splenectomy is advocated the phosphorylation of band 2 is depressed by approx. 70%. After splenectomy the phosphorylation of membrane proteins is restored to normal levels.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D000242:Cyclic AMP / Q000494:pharmacology; D004591:Electrophoresis, Polyacrylamide Gel; D004910:Erythrocyte Membrane / Q000378:metabolism*; D004912:Erythrocytes / Q000378:metabolism*; D005260:Female; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism*; D010455:Peptides / Q000097:blood; D010710:Phosphates / Q000378:metabolism*; D010766:Phosphorylation; D013049:Spectrin / Q000378:metabolism*; D013103:Spherocytosis, Hereditary / Q000097:blood* / Q000601:surgery; D013156:Splenectomy
D008565:Membrane Proteins; D010455:Peptides; D010710:Phosphates; D013049:Spectrin; D000242:Cyclic AMP