Faculté de Médecine & Pharmacie de Rouen, Rouen cedex, France.
Hamostaseologie. 2011 Aug;31(3):155-8, 160-4. doi: 10.5482/ha-1161. Epub 2011 Jun 21.
Protein Z (PZ) is a vitamin K-dependent factor identified in human plasma in 1984 but it has no enzymatic activity. It is a cofactor of a serpin, the protein Z-dependent protease inhibitor (ZPI), and the complex PZ/ZPI inhibits activated factor X on phospholipid surfaces. In mice, the disruption of PZ or ZPI gene is asymptomatic, but enhances the thrombotic phenotype and mortality of other thrombotic risk factors. Most of the clinical studies focused on PZ. Despite conflicting results, a recent meta-analysis indicated that PZ deficiency could be a risk for venous and arterial thrombosis and early fetal loss. However, these conclusions are drawn from case-control studies of small size, constituting an important limitation. Recently, it was shown that PZ and/or ZPI are synthesised by normal kidney and different cancer cells, suggesting that the complex PZ/ZPI could play a role in inhibiting the tissue deposition of fibrin. The physiopathological consequences of these observations remain to be established. At this time, the measurement of plasma PZ and ZPI or analysis of their gene polymorphisms should not be performed routinely for the exploration of thrombophilia.
蛋白 Z（PZ）是 1984 年在人血浆中发现的一种维生素 K 依赖性因子，但它没有酶活性。它是丝氨酸蛋白酶抑制剂（ZPI）的一个辅助因子，该复合物 PZ/ZPI 抑制磷脂表面上的活化因子 X。在小鼠中，PZ 或 ZPI 基因的缺失无明显症状，但增强了其他血栓形成风险因素的血栓形成表型和死亡率。大多数临床研究都集中在 PZ 上。尽管结果存在矛盾，但最近的荟萃分析表明，PZ 缺乏可能是静脉和动脉血栓形成以及早期胎儿丢失的风险因素。然而，这些结论是从规模较小的病例对照研究中得出的，这构成了一个重要的局限性。最近表明，PZ 和/或 ZPI 由正常肾脏和不同的癌细胞合成，这表明 PZ/ZPI 复合物可能在抑制纤维蛋白组织沉积中发挥作用。这些观察结果的生理病理后果仍有待确定。此时，不应该常规进行血浆 PZ 和 ZPI 的测量或分析其基因多态性，以探索血栓形成倾向。
