Association of decreased membrane protein phosphorylation with red blood cell spherocytosis.

A close association between sphering of human red cells and deficient phosphorylation of their membrane proteins has been documented in three spearate situations. Red cells rendered spheroidal by exposure to: (1) elevated temperatures; (2) two sulfhydryl inhibitors (N-ethylmaleimide or paramercuribenzoate); or (3) in the genetic situation of hereditary spherocytosis--all manifest deficient phosphorylation of endogenous membrane proteins by ATP. In the two former cases, we have noted an exact association between the onset of red cell sphering (e.g., as temperatures rose above 48 degrees C or N-ethylmaleimide concentrations exceeded 2 mumoles/ml RBC) and the development of deficient ghost protein phosphorylation.