Deficiency of the complement regulatory protein, "decay-accelerating factor," on membranes of granulocytes, monocytes, and platelets in paroxysmal nocturnal hemoglobinuria.
Erythrocytes from patients with paroxysmal nocturnal hemoglobinuria are deficient in decay-accelerating factor, a membrane protein that inhibits the complement C3 convertases. We studied the expression of this protein on leukocytes and platelets from four patients with paroxysmal nocturnal hemoglobinuria, using cytofluorographic analysis and antibody to decay-accelerating factor. The granulocytes and monocytes had a bimodal distribution of fluorescence, indicating antigen-deficient and antigen-positive subpopulations of cells. In contrast, granulocytes and monocytes from normal donors and patients with other diseases had no antigen-deficient cells. Platelets from the four patients with paroxysmal nocturnal hemoglobinuria had less fluorescence than normal platelets. Furthermore, surface-radiolabeled granulocytes and platelets from one of the four patients, which were maximally deficient in decay-accelerating factor, also lacked antigen that was immunoprecipitable by specific antibody to this protein. Thus, paroxysmal nocturnal hemoglobinuria is a clonal disorder characterized by deficient membrane expression of decay-accelerating factor on granulocytes, monocytes, and platelets, as well as on erythrocytes.
D000328:Adult; D001792:Blood Platelets / Q000276:immunology*; D003166:Complement Activating Enzymes / Q000037:antagonists & inhibitors*; D050577:Complement C3-C5 Convertases / Q000037:antagonists & inhibitors*; D003171:Complement Pathway, Classical; D005260:Female; D005434:Flow Cytometry; D006098:Granulocytes / Q000276:immunology*; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000276:immunology*; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000172:deficiency* / Q000276:immunology; D008875:Middle Aged; D009000:Monocytes / Q000276:immunology*
D008565:Membrane Proteins; D003166:Complement Activating Enzymes; D050577:Complement C3-C5 Convertases