Deficiency of a Granulocyte-Membrane Glycoprotein (gp150) in a Boy with Recurrent Bacterial Infections

Abstract

Investigation of the basis for increased susceptibility to infection with pyogenic organisms in an eight-year-old boy revealed a defect in receptor coupled polymorphonuclear-leukocyte functions. For example, phagocytosis of opsonized oil red O particles or particles coated with immunoglobulin, superoxide generation, and degranulation induced by opsonized zymosan were decreased. This defect in receptor-coupled function was not due to decreased receptor–ligand binding or to impaired superoxide generation or degranulation when these effects were elicited by soluble stimuli. The defect appeared to be associated with the absence of a normal granulocyte-surface-membrane glycoprotein with an approximate molecular weight of 150,000 daltons (gp150). This glycoprotein was present in reduced amounts (approximately 50 per cent of normal) in the granulocytes from each of the patient's parents, suggesting a genetic basis for its deficiency. (N Engl J Med. 1982; 306:693–9.)