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. 2010 Winter;19(4):e120-5.
doi: 10.1055/s-0031-1278382.

Participation of protein Z-dependent protease inhibitor and protein Z system in the pathomechanism of thrombotic complications

Małgorzata A Gacka  1 , Rafał Małecki, Rajmund Adamiec
Affiliations

Participation of protein Z-dependent protease inhibitor and protein Z system in the pathomechanism of thrombotic complications

Małgorzata A Gacka et al. Int J Angiol. 2010 Winter.

Abstract

Thrombotic complications of unknown etiology remain a serious diagnostic and therapeutic problem. Occurrence of the inherited polymorphisms of genes encoding proteins involved in the coagulation cascade is one of the possible causes of these complications. In recent years, protein Z (PZ) and PZ-dependent protease inhibitor (ZPI) have been added to the list of prothrombotic factors. PZ is a glycoprotein serving as a cofactor of ZPI, which is responsible for the inhibition of prothrombinase. Expression of the PZ gene is under the control of many transcriptional factors; several polymorphisms alternate the rate of gene expression. The present article describes the significance of the ZPI-PZ system in venous and arterial thrombosis, adverse pregnancy outcomes and antiphospholipid syndrome complications.

Keywords: Adverse pregnancy outcomes; Arterial thrombosis; Protein Z; Protein Z-dependent protease inhibitor; Venous thrombosis.

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Figures

Figure 1)
Figure 1)
A complex consisting of activated factors X (Xa) and V (Va), membrane phospholipids and calcium ions (prothrombinase) forms the last step of the coagulation cascade preceding the conversion of prothrombin into thrombin. Prothrombinase can be activated directly by factor VIIa and tissue factor (TF; extrinsic pathway) or through activated factors XIIa, XIa, IXa and VIIIa (intrinsic pathway). Two serine protease systems (serpins) are mainly responsible for the inactivation of prothrombinase – antithrombin (AT; physiologically activated by heparan sulfate) and protein Z (PZ)-dependent protease inhibitor (ZPI), which forms a complex with PZ. G Glycosaminoglycan (eg, heparan sulfate)
See this image and copyright information in PMC

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