Protein Z influences the prothrombotic phenotype in Factor V Leiden patients

Kemkes‐Matthes, B.; Nees, Matthias; Kühnel, Gitta; Matzdorff, Axel; Matthes, K.

doi:10.1016/s0049-3848(02)00181-0

Cited by 79 publications

(95 citation statements)

References 11 publications

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“…We recently described a P2Y 12 gene polymorphism associated with ADPinduced maximal platelet aggregation [3], the H2 haplotype being associated with a higher aggregation response in healthy subjects. Moreover, we found a significant association between the P2Y 12 H2 haplotype and peripheral vascular disease in a case-control study [4]. In view of the results presented by Leon et al, we hypothesized that the H1/H2 polymorphism could be associated with collagen-induced platelet aggregation response, basal TF expression in monocytes (TF mRNA), plasma…”

Section: )1mentioning

confidence: 51%

“…The PZdependent protease inhibitor in the presence of PZ causes fast inactivation of factor Xa. Recently, reduced circulating levels of PZ have been suggested to play a role in the occurrence of bleeding [2] and deep vein thrombosis [3,4], as well as primary aborts with unexplained early fetal loss [5]. However, these data have been disputed [6][7][8].…”

Section: R S a N T A C R O C E F C A P P U C C I * P D I P mentioning

confidence: 99%

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Protein Z gene polymorphisms are associated with protein Z plasma levels

Santacroce

Cappucci

et al. 2004

Journal of Thrombosis and Haemostasis

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Section: )1mentioning

confidence: 51%

Section: R S a N T A C R O C E F C A P P U C C I * P D I P mentioning

confidence: 99%

Protein Z gene polymorphisms are associated with protein Z plasma levels

Santacroce

Cappucci

et al. 2004

Journal of Thrombosis and Haemostasis

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“…ZPI also inhibits factor XIa efficiently independent of protein Z (3,4). The importance of ZPI and PZ as anticoagulant regulators of factor Xa and factor XIa is suggested by the prothrombotic phenotype of PZ knock-out mice and embryonic lethality of ZPI knock-out mice harboring the factor V Leiden mutation (5,6), consistent with the aggravated thromboembolic risk observed in factor V Leiden patients with PZ deficiency (7). Clinical studies concerning the relationship between ZPI/PZ levels and risk of venous thrombosis and thromboembolic diseases are often conflicting, although generally there are more reports supporting such association than those that do not (8,9).…”

Section: Protein Z-dependent Protease Inhibitor (Zpi)mentioning

confidence: 61%

Thermodynamic and Kinetic Characterization of the Protein Z-dependent Protease Inhibitor (ZPI)-Protein Z Interaction Reveals an Unexpected Role for ZPI Lys-239

Huang

Zhou

et al. 2015

Journal of Biological Chemistry

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Background: ZPI-protein Z complex is a critical anticoagulant regulator of membrane-associated factor Xa. Results: The energetics and multistep kinetics of the ZPI-protein Z interaction were characterized with fluorescently labeled K239C ZPI. Conclusion: Ionic and hydrophobic interactions mediate ZPI-protein Z binding, and conserved ZPI Lys-239 promotes protein Z dissociation. Significance: The studies establish the thermodynamic and kinetic basis for catalytic activation of ZPI by protein Z.

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“…11 Patients with homozygotic factor-V-disease mutation, however, combine low Protein-Z levels with an increased procoagulation potential. 12 These examples may shed some light on the ambivalent function of some factors in many coagulation defects caused by the inherent complexity of the regulation systems.…”

Section: Discussionmentioning

confidence: 99%

Protein-Z-deficiency as a rare case of unexpected perioperative bleeding in a patient with spinal cord injury

Röhl

Jäger²,

Barth

2006

Spinal Cord

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Study design: Case report describing the management of repeated perioperative bleeding probably due to Protein-Z-deficiency in a post-traumatic paraplegic patient. Objectives: To describe the difficulty in diagnosing this rare form of hypocoagulability and the monitoring and substitution concept during three elective surgical interventions. Setting: Spinal Cord Injury Center, Bergmannstrost, Halle, Germany. Case report: A 19-year-old male suffering from a post-traumatic paraplegia sub Th8 (ASIA-A) since childhood had experienced two life-threatening intraoperative bleeding incidents before finally Protein-Z-deficiency as the underlying coagulation disorder was diagnosed. After substitution of 2000 IE PPSB (Beriplex P/N) a repeatedly postponed implantation of a sphincter-externus (Brindley-) stimulator could be performed without bleeding complications, and this was also true for two additional urological interventions 1 year later. Protein-Z levels were monitored before, during and after the operations. The preoperative application of between 1000 and 2000 IE PPSB was safe and sufficient to raise the patients' plasma Protein-Z level to almost normal and so prevent excessive intraoperative blood loss. Conclusion: In case of repeated bleeding tendency of unknown origin it is mandatory to look for rare causes of hypocoagulability such as Protein-Z-deficiency. We developed a substitution concept using a plasma concentrate with guaranteed Protein-Z amount (PPSB) allowing the safe performance of elective surgical interventions.

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Protein Z influences the prothrombotic phenotype in Factor V Leiden patients

Cited by 79 publications

References 11 publications

Protein Z gene polymorphisms are associated with protein Z plasma levels

Protein Z gene polymorphisms are associated with protein Z plasma levels

Thermodynamic and Kinetic Characterization of the Protein Z-dependent Protease Inhibitor (ZPI)-Protein Z Interaction Reveals an Unexpected Role for ZPI Lys-239

Protein-Z-deficiency as a rare case of unexpected perioperative bleeding in a patient with spinal cord injury

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