Gardiner J E, Griffin J H
Thromb Res. 1984 Nov 1;36(3):197-203. doi: 10.1016/0049-3848(84)90219-6.
Activated protein C is a potent anticoagulant which inactivates Factors V and VIII in plasma. Normal plasma contains an inhibitor of activated protein C. A previous report from our laboratory hypothesized that the absence of this inhibitor in plasmas from patients with combined Factor V/VIII deficiency could be the molecular basis for this disease. This report demonstrates the presence of protein C inhibitor in those Factor V/VIII deficient plasmas originally studied. Freezing and thawing significantly reduced the ability of normal and Factor V/VIII deficient plasmas to inhibit activated protein C. It is suggested that this explains the conflicting literature reports describing functional assays of protein C inhibitor in Factor V/VIII deficient plasma. This observation also emphasizes that extreme care must be used in handling and storing plasma samples that are to be assayed for protein C inhibitor.
活化蛋白C是一种强效抗凝剂，可使血浆中的因子V和因子VIII失活。正常血浆含有活化蛋白C的抑制剂。我们实验室之前的一份报告推测，联合因子V/因子VIII缺乏症患者血浆中缺乏这种抑制剂可能是该疾病的分子基础。本报告证明，在最初研究的那些因子V/因子VIII缺乏的血浆中存在蛋白C抑制剂。冻融显著降低了正常血浆和因子V/因子VIII缺乏血浆抑制活化蛋白C的能力。这表明，这解释了文献中关于因子V/因子VIII缺乏血浆中蛋白C抑制剂功能测定的相互矛盾的报告。这一观察结果还强调，在处理和储存用于检测蛋白C抑制剂的血浆样本时必须格外小心。
