MECHANISM OF COMPLEMENT-MEDIATED ACTIVATION OF HUMAN-BLOOD PLATELETS INVITRO - COMPARISON OF NORMAL AND PAROXYSMAL NOCTURNAL HEMOGLOBINURIA PLATELETS
MECHANISM OF COMPLEMENT-MEDIATED ACTIVATION OF HUMAN-BLOOD PLATELETS INVITRO - COMPARISON OF NORMAL AND PAROXYSMAL NOCTURNAL HEMOGLOBINURIA PLATELETS
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DOI：
10.1172/jci108648
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发表时间：
1977-01-01
影响因子：
15.9
通讯作者：
ROSSE, WF
中科院分区：
文献类型：
--
作者：
DIXON, RH;ROSSE, WF
阵发性睡眠性血红蛋白尿症（PNH）血小板与正常人血小板的不同之处在于其与活化补体[C]组分的相互作用：当补体被旁路途径活化时，与正常血小板相比，更多的C3固定在PNH血小板上。血小板释放反应，如通过5-羟色胺释放测量的，发生在C3固定到PNH血小板后。正常血小板不会发生这种反应。虽然单独由抗体介导的5-羟色胺释放对于正常和PNH血小板是相同的，但抗体引发的补体激活导致更大量的C3固定到PNH血小板和更大的随后5-羟色胺释放。几乎最大的5-羟色胺从PNH血小板释放发生在C3（或可能C5）固定到膜上后，而没有完成末端序列。相反，完成C5以外的末端补体序列是正常血小板释放最大5-羟色胺所必需的。这些补体成分和PNH血小板相互作用的异常可能解释了这种疾病中血栓形成的发生。
The paroxysmal nocturnal hemoglobinuria (PNH) platelet differs from the normal human platelet in its interaction with activated complement[C]components: when complement is activated by the alternative pathway, greater amounts of C3 are fixed to the PNH platelet than to the normal platelet. The platelet-release reaction, as measured by serotonin release, occurs after C3 fixation to the PNH platelet. This reaction does not occur with normal platelets. Although serotonin release mediated by antibody alone was the same for normal and PNH platelets, antibody-initiated complement activation resulted in the fixation of greater amounts of C3 to PNH platelets and greater consequent serotonin release. Nearly maximal serotonin release from PNH platelets occurs after the fixation of C3 (or perhaps C5) to the membrane without completion of the terminal sequence. In contrast, completion of the terminal complement sequence beyond C5 is required for maximal serotonin release from normal platelets. These abnormalities of interaction of complement components and PNH platelets may explain the occurrence of thromboses in this disease.