Protein z deficiency

Common Name(s)

Protein z deficiency

Protein Z serves as a cofactor for the downregulation of coagulation by forming a complex with the protein Z-dependent protease inhibitor (ZPI; {605271}). Evidence also suggests that protein Z may promote the assembly of thrombin with phospholipid surfaces, thus enhancing coagulation. There is a wide variation of protein Z levels in human plasma, and studies have reported conflicting results of the clinical consequences of protein Z deficiency in humans. Most studies have reported an association between decreased protein Z levels and thrombosis, including stroke, venous thrombosis, and obstetric complications, although early reports suggested an association between protein Z deficiency and bleeding tendency ({4:Kemkes-Matthes and Matthes, 1995}). Overall, a role for protein Z in the pathogenesis of hemostatic disorders in humans is controversial and remains unclear (review by {1:Broze, 2001} and {11:Vasse, 2008}). Protein Z deficiency has been shown to exacerbate the thrombotic phenotype in patients with thrombophilia due to factor V Leiden (see {188055}).
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Protein z deficiency" for support, advocacy or research.

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Protein z deficiency" returned 6 free, full-text research articles on human participants. First 3 results:

Protein Z-deficiency is associated with enhanced neointima formation and inflammatory response after vascular injury in mice.
 

Author(s): Antje Butschkau, Nana-Maria Wagner, Laura Bierhansl, Berit Genz, Brigitte Vollmar

Journal:

 

Protein Z (PZ) is a vitamin K-dependent coagulation factor without catalytic activity. Evidence points towards PZ as an independent risk factor for the occurrence of human atherosclerotic vascular diseases. The aim of this study was to investigate the role of PZ in vascular arterial disease.

Last Updated: 31 Dec 1969

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Pyelonephritis during pregnancy: a cause for an acquired deficiency of protein Z.
 

Author(s): Jyh Kae Nien, Roberto Romero, Debra Hoppensteadt, Offer Erez, Jimmy Espinoza, Eleazar Soto, Juan Pedro Kusanovic, Francesca Gotsch, Chong Jai Kim, Pooja Mittal, Jawed Fareed, Joaquin Santolaya, Tinnakorn Chaiworapongsa, Samuel Edwin, Beth Pineles, Sonia Hassan

Journal: J. Matern. Fetal. Neonatal. Med.. 2008 Sep;21(9):629-37.

 

Pyelonephritis has a more severe course during pregnancy than in the non-pregnant state. This has been attributed to the increased susceptibility of pregnant women to microbial products. An acquired protein Z deficiency has been reported when there is excessive thrombin activity. ...

Last Updated: 31 Dec 1969

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An additional Glu30Lys substitution in the Gla domain of the protein Z gene is not a common polymorphism but a rare mutation, which would cause its deficiency.
 

Author(s): H Iwata, M Souri, B Kemkes-Matthes, A Ichinose

Journal: J. Thromb. Haemost.. 2005 Oct;3(10):2360-1.

 

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

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The terms "Protein z deficiency" returned 0 free, full-text review articles on human participants.

 
 
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