Autoimmune antibody (IgG Kansas) against the fibrin stabilizing factor (factor XIII) system.
Autoimmune antibody (IgG Kansas) against the fibrin stabilizing factor (factor XIII) system.
复制标题
针对纤维蛋白稳定因子（因子 XIII）系统的自身免疫抗体（IgG Kansas）。
DOI：
10.1073/pnas.85.1.232
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发表时间：
1988
影响因子：
11.1
通讯作者：
Zucker,ML
中科院分区：
文献类型：
--
作者：
Lorand,L;Velasco,PT;Rinne,JR;Amare,M;Miller,LK;Zucker,ML
一名85岁的获得性出血性疾病发作后4个月内死于大出血的患者的血清中含有一种有效的纤维蛋白稳定抑制剂。其他凝血、纤溶参数及出血时间均在正常范围内。该抑制剂显示为具有κ轻链的IgG（IgG堪萨斯）;其特异性靶点为因子XIII系统本身。尽管IgG堪萨斯与酶原的原始[ab]形式结合，但它不阻断凝血酶催化的转化为[a 'b]。然而，IgG堪萨斯阻止了随后的Ca 2+介导的[a 'B]活化为a + B，其中a表示催化活性因子XIIIa种类。IgG堪萨斯，与以前研究的类似出血性疾病的自身免疫抗体（IgG华沙）相反，也可以抑制预活化的a酶的转酰胺活性。
Serum from a patient who died from massive hemorrhage within 4 months after onset of an acquired bleeding disorder at age 85 contained a potent inhibitor of fibrin stabilization. Other parameters of coagulation and fibrinolysis and his bleeding time were within normal limits. The inhibitor was shown to be an IgG with kappa light chains (IgG Kansas); its specific target was the factor XIII system itself. Although IgG Kansas combined with the virgin [ab] form of the zymogen, it did not block the thrombin-catalyzed conversion to [a'b]. However, IgG Kansas prevented the subsequent Ca2+-mediated activation of [a'b] to a + b, where a denotes the catalytically competent factor XIIIa species. IgG Kansas, in contrast to a previously studied autoimmune antibody from a similar bleeding disorder (IgG Warsaw), could also inhibit the transamidating activity of the preactivated a enzyme.