Dysfunctional Activated Protein C (PC Cádiz) in a Patient with Thrombotic Disease
Dysfunctional Activated Protein C (PC Cádiz) in a Patient with Thrombotic Disease
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血栓性疾病患者的功能失调性活化蛋白 C (PC Cádiz)
DOI：
10.1055/s-0038-1651090
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发表时间：
1987
影响因子：
6.7
通讯作者：
M. Rutllant
中科院分区：
文献类型：
--
作者：
N. Sala;M. Borrell;K. Bauer;S. Vigano'D'Angelo;J. Fontcuberta;Jordi Félez;M. Rutllant
总结描述了一名患有复发性静脉血栓形成的45岁男性患者中功能失调的蛋白C（PC）（暂时命名为“PC加的斯”）的部分特征。在止血和肝功能的实验室测定中发现的唯一缺陷是通过使用凝血酶-血栓调节蛋白复合物和蛇毒激活蛋白C的不同功能测定测量的酰胺分解和抗凝蛋白C活性的一半正常水平。蛋白C抗原始终在正常水平范围内。先证者的两个年幼的女儿被发现有同样的缺陷。双交叉免疫电泳，在存在和不存在的Ca 2+在第一个维度，显示患者和对照PC之间没有明显的差异。PC吸附钡盐也被发现是正常的。PC激活后的柠檬酸钡洗脱液中的PC激活肽的测量显示，患者和10名正常对照之间没有显着差异，该肽的浓度是非常相似的PC酶原在PC激活前相同的洗脱液。这些结果表明，这种异常的PC能够被凝血酶-血栓调节蛋白复合物正常激活，但不表现出丝氨酸蛋白酶活性，可能是由于活性位点中心附近的PC分子中的缺陷。
Summary The partial characterization of a dysfunctional protein C (PC), provisionally named “PC Cádiz”, in a 45-year-old male patient suffering from recurrent venous thrombosis is described. The only defect found in laboratory assays for haemostasis and hepatic function was a half normal level of both amidolytic and anticoagulant protein C activity, measured by different functional assays that use thrombin-thrombomodulin complex and a snake venom to activate protein C. Protein C antigen was always found to be within normal levels. Two young daughters of the propositus were found to have the same defect. Double-crossed immunoelectrophoresis, performed in the presence and absence of Ca2+ in the first dimension, showed no clear differences between patient and control PC. PC adsorption to barium salts was also found to be normal. Measurement of the PC activation peptide in the barium citrate eluates after PC activation showed no significant differences between patient and 10 normal controls, the concentration of this peptide being very similar to that of PC zymogen in the same eluates before PC activation. These results indicate that this abnormal PC is able to be normally activated by thrombin-thrombomodulin complex but does not exhibit serine protease activity, probably due to a defect in the PC molecule near the active site center.
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蛋白 S 是活化蛋白 C 中和血小板释放的纤溶酶原激活抑制剂的辅助因子。
影响因子：
20.3
作者：
D'Angelo,A;Lockhart,MS;D'Angelo,SV;TaylorJr,FB
通讯作者：
TaylorJr,FB
DOI：
10.1016/s0021-9258(19)68566-2
发表时间：
1981-11
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
F. Walker
通讯作者：
F. Walker
口服抗凝期间和选定疾病状态下蛋白 C 抗原与抗凝活性之间的关系。
DOI：
10.1172/jci112319
发表时间：
1986
期刊：
The Journal of clinical investigation
影响因子：
--
作者：
ViganoD'Angelo,S;Comp,PC;Esmon,CT;D'Angelo,A
通讯作者：
D'Angelo,A
DOI：
10.1073/pnas.78.4.2249
发表时间：
1981-01-01
期刊：
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES
影响因子：
--
作者：
ESMON, CT;OWEN, WG
通讯作者：
OWEN, WG