GLYCOPROTEIN-180 DEFICIENCY - GENETICS AND ABNORMAL NEUTROPHIL ACTIVATION

  • 1 January 1985
    • journal article
    • research article
    • Vol. 65  (3) , 696-704
Abstract
Neutrophil function was studied in a patient with polymorphonuclear leukocyte (PMN) glycoprotein-180 deficiency and in her parents. PMN of the patient had abnormal chemotaxis, phagocytosis, adherence, surface charge and membrane-associated events of activation. Selective defects to C3b, IgG, phorbol myristate acetate (PMA) and N-for-Met-Leu-Phe (FMLP) are described, although C3b receptor density was normal. The parents were found to have abnormal adherence to nylon-wool fibers, abnormal transmembrane potential depolarization with PMA, and reduced amounts of glycoprotein-180 in their PMN. The oxidative burst has several different pathways for activation. The absence of a single PMN surface glycoprotein is associated with a broad spectrum of PMN functional abnormalities. Finally, the observations made in the parents support an autosomal recessive mode of inheritance.