نتایج جستجو برای: fxiii deficiency

تعداد نتایج: 137316  

Journal: :Physiological reviews 2011
László Muszbek Zsuzsanna Bereczky Zsuzsa Bagoly István Komáromi Éva Katona

Factor XIII (FXIII) is unique among clotting factors for a number of reasons: 1) it is a protransglutaminase, which becomes activated in the last stage of coagulation; 2) it works on an insoluble substrate; 3) its potentially active subunit is also present in the cytoplasm of platelets, monocytes, monocyte-derived macrophages, dendritic cells, chondrocytes, osteoblasts, and osteocytes; and 4) i...

Journal: :archives of medical laboratory sciences 0
majid naderi professor; departmetn of virology, tarbiat modares university, tehran samira esmaeili-reykande shaban alizadeh akbar dorgalaleh zahra sadat abtahi zahra kashani-khatib

background: factor xiii deficiency (fxiiid) is an inherited rare bleeding disorder with some life threatening clinical manifestation including intracranial haemorrhage (ich). among all polymorphisms found in fxiiid, thrombin activatable fibrinolysis inhibitor (tafi) thr325ile gene polymorphism increases probability ofichabout 20 fold in patients with fxiii .so, in this study we aimed to evaluat...

Journal: :European heart journal 2008
Davy Vanhoutte Stephane Heymans

Heart failure (HF) affects .11 million people in the USA and Europe, and ischaemic heart disease accounts for .60% of these patients. Despite excellent diagnostic and therapeutic standards for myocardial infarction (MI), improved early phase imaging and therapy are essential to prevent the progression of cardiac dilatation and dysfunction after acute MI. Transglutaminases (TGs) are a widely dis...

2013
US Schött DW Winstedt JH Hanna

Objectives: Natural colloid albumin induces a lesser degree of dilutional coagulopathy than synthetic colloids. Fibrinogen concentrate has emerged as a promising strategy to treat coagulopathy, and factor XIII (FXIII) works synergistically with fibrinogen to correct coagulopathy following haemodilution with crystalloids. Objectives were to examine the ability of fibrinogen and FXIII concentrate...

Journal: :Thrombosis and haemostasis 2015
Gerhard Dickneite Heiko Herwald Wolfgang Korte Yannick Allanore Christopher P Denton Marco Matucci Cerinic

Coagulation factor XIII (FXIII), a plasma transglutaminase, is best known as the final enzyme in the coagulation cascade, where it is responsible for cross-linking of fibrin. However, a growing body of evidence has demonstrated that FXIII targets a wide range of additional substrates that have important roles in health and disease. These include antifibrinolytic proteins, with cross-linking of ...

Journal: :Diabetes 2002
Kristina F Standeven Robert A S Ariëns Paul Whitaker Alison E Ashcroft John W Weisel Peter J Grant

The antihyperglycemic drug dimethylbiguanide (DMB, also known as metformin) reduces the risk of cardiovascular complications in type 2 diabetes, although the mechanism(s) involved are unclear. DMB reduces glycosylation-related protein cross-linking, a process similar to fibrin cross-linking catalyzed by activated factor XIII (FXIII). To investigate whether the cardioprotective effect of DMB cou...

Journal: :Blood 2000
I Balogh G Szôke L Kárpáti U Wartiovaara E Katona I Komáromi G Haramura G Pfliegler H Mikkola L Muszbek

Val34Leu polymorphism of the A subunit of coagulation factor XIII (FXIII-A) is located in the activation peptide (AP) just 3 amino acids away from the thrombin cleavage site. This mutation has been associated with a protective effect against occlusive arterial diseases and venous thrombosis; however, its biochemical consequences have not been explored. In the current study it was demonstrated t...

2006
Philip S. Wells Josdalyne L. Anderson Dimitrios K. Scarvelis Steve P. Doucette France Gagnon

It has been suggested that a G-to-T transition in exon 2 of the factor XIIIA gene resulting in a substitution of leucine for valine at amino acid 34 (FXIII Val34Leu) protects against venous thromboembolism (VTE). However, the evidence to date is insufficient to incorporate testing for the FXIII Val34Leu variant into clinical practice. To determine whether genotypes with the FXIII Val34Leu varia...

2010
N J Mutch J S Koikkalainen S R Fraser K M Duthie M Griffin J Mitchell H G Watson N A Booth

BACKGROUND Activated factor XIII (FXIIIa), a transglutaminase, introduces fibrin-fibrin and fibrin-inhibitor cross-links, resulting in more mechanically stable clots. The impact of cross-linking on resistance to fibrinolysis has proved challenging to evaluate quantitatively. METHODS We used a whole blood model thrombus system to characterize the role of cross-linking in resistance to fibrinol...

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