DEFICIENCY OF THE COMPLEMENT REGULATORY PROTEIN, DECAY-ACCELERATING FACTOR, ON MEMBRANES OF GRANULOCYTES, MONOCYTES, AND PLATELETS IN PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA
DEFICIENCY OF THE COMPLEMENT REGULATORY PROTEIN, DECAY-ACCELERATING FACTOR, ON MEMBRANES OF GRANULOCYTES, MONOCYTES, AND PLATELETS IN PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA
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DOI：
10.1056/nejm198504253121704
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发表时间：
1985-01-01
影响因子：
158.5
通讯作者：
AUSTEN, KF
中科院分区：
文献类型：
--
作者：
NICHOLSONWELLER, A;SPICER, DB;AUSTEN, KF
Erythrocytes from patients with paroxysmal nocturnal hemoglobinuria were deficient in decay-accelerating factor, a membrane protein that inhibits the complement C3 convertases. The expression of this protein on leukocytes and platelets from 4 patients with paroxysmal nocturnal hemoglobinuria, was studied 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. Granulocytes and monocytes from normal donors and patients with other diseases had no antigen-deficient cells. Platelets from the 4 patients with paroxysmal nocturnal hemoglobinuria had less fluorescence than normal platelets. Surface-radiolabeled granulocytes and platelets from 1 of the 4 patients, which were maximally deficient in decay-accelerating factor, also lacked antigen that was immunoprecipitable by specific antibody to this protein. 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.
