Functional analysis of protein Z (Arg255His) and protein Z‐dependent protease inhibitor (Lys25Arg and Ser40Gly) polymorphisms

Ndonwi, Matthew; Lu, Lan; Tu, Yizheng; Phillips, Michelle; Broze, George J.

doi:10.1111/j.1365-2141.2008.07322.x

Cited by 8 publications

(3 citation statements)

References 11 publications

(18 reference statements)

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“…PZ is a plasma vitamin K-dependent glycoprotein acting as a cofactor of ZPI to inhibit factor Xa together with Ca 2+ and phospholipid [17]. Recent studies have represented evidences on the potential role of this protein in thromboembolic complications [13].…”

Section: Discussionmentioning

confidence: 99%

Plasma Concentrations of Protein Z and Protein Z-Dependent Protease Inhibitor in Thalassemia Major Patients

Ghazanfari

Far

Shirali

et al. 2019

SN Compr. Clin. Med.

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Thalassemia is the most common congenital hemolytic disorder by a partial or complete deficiency in globin chain synthesis. Recent investigations have suggested a correlation between protein Z (PZ) and protein Z-dependent protease inhibitor (ZPI) deficiency and thrombosis. So, the aim of this study was to evaluate the PZ and ZPI levels in beta (β)-thalassemia patients. In this study, 40 patients with thalassemia major and 40 control subjects were selected. PZ and ZPI serum levels were measured by sandwich ELISA technique. PZ and ZPI were significantly higher in thalassemic patient group than control group. Also, PZ levels and PLT and Hb counts were significantly higher in splenectomized thalassemic patients than non-splenectomized ones. The exact mechanism of increased PZ and ZPI have an impact on hypercoagulable state in β-thalassemia major could not be found in our study. More studies are still needed to understand these challenges in β-thalassemia major patients.Keywords Thalassemia major . Protein Z . Protein Z-dependent protease inhibitor . Plasma . Coagulability state Highlights • PZ and ZPI levels were significantly increased in β-thalassemia major group than control group • PZ levels, PLT and Hb counts were significantly increased in splenectomized thalassemic patients than non-splenectomized • PZ and ZPI increased levels did not show the exact influence on coagulability in thalassemia major patients This article is part of the Topical Collection on Medicine.

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Section: Discussionmentioning

confidence: 99%

Plasma Concentrations of Protein Z and Protein Z-Dependent Protease Inhibitor in Thalassemia Major Patients

Ghazanfari

Far

Shirali

et al. 2019

SN Compr. Clin. Med.

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“…Perhaps the rare genotypes could modify also the PZ activity. To prove it, it will be necessary to have cDNA constructs for polymorphic forms of PZ and to measure the "functional" activity of these constructs in an "in vitro" system as described by Nondwy et al (37).…”

Section: Discussionmentioning

confidence: 99%

Rare genotypes of protein Z gene are a risk factor for premature myocardial infarction but not protein Z plasma level

Cam-Duchez

Soria²,

Sollier³

et al. 2009

Thromb Haemost

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Protein Z (PZ) is the cofactor of PZ dependent inhibitor (ZPI) that inhibits activated coagulation factor X. PZ was expected to play a role in coronary artery disease (CAD) but with inconsistent clinical findings. We therefore evaluated whether PZ plasma level and/or three genetic variants encoding for low PZ plasma level were associated with premature CAD in stable young post-myocardial infarction (MI) patients. PZ plasma level and three polymorphisms A-13G, G-103A and G79A were determined in 176 young stable post-MI patients and in 176 sex- and age-matched controls (FITE-NAT population). Moreover the genotypes, resulting from the combination of the three polymorphisms (A-13G/G-103A/G79A), were studied. PZ plasma level and the number of patients disclosing a PZ deficiency did not differ between post-MI patients and controls. The presence of the mutated allele for each polymorphism was associated with a significantly reduced level of PZ. The A-13G polymorphism was associated with premature CAD only in univariate analysis. Whereas, the presence of rare genotypes of PZ gene was an independent risk factor for premature CAD. In conclusion, PZ plasma level is not a key player in the pathophysiology of premature coronary artery disease. But, rare genotypes of PZ gene were found to be associated with premature CAD.

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“…Another polymorphism, which may worsen the course of VTE among factor V Leiden carriers, is the substitution of arginine by histidine in locus 255 within the pseudocatalytic domain. A similar role may be played by the coexistence of Lys25Arg and Ser40Gly polymorphisms although in vitro studies did not confirm any difference between these substitutions and wild variants (29). From a theoretical point of view, the impact of PZ on thrombosis may be indirect and may depend on ZPI function.…”

Section: Pz and Venous Thromboembolic Riskmentioning

confidence: 94%

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

Gacka

Małecki²,

Adamiec³

2010

Int J Angiol

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Functional analysis of protein Z (Arg255His) and protein Z‐dependent protease inhibitor (Lys25Arg and Ser40Gly) polymorphisms

Abstract: Minimal residual disease may predict bone marrow relapse in patients with hairy cell leukemia treated with 2-chlorodeoxyadenosine. Blood, 87, 1556-1560.

Cited by 8 publications

References 11 publications

Plasma Concentrations of Protein Z and Protein Z-Dependent Protease Inhibitor in Thalassemia Major Patients

Plasma Concentrations of Protein Z and Protein Z-Dependent Protease Inhibitor in Thalassemia Major Patients

Rare genotypes of protein Z gene are a risk factor for premature myocardial infarction but not protein Z plasma level

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

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