AN UPDATED CLASSIFICATION OF FACTOR XIII DEFECT

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Factor XIII deficiency: a review of literature

Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...

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Although many epidemiologic studies have investigated the FXIII-A Val34Leu polymorphism and their associations with myocardial infarction (MI), definite conclusions can't be drawn. To clarify the effects of FXIII-A Val34Leu polymorphism on the risk of MI, a meta-analysis was performed. Related studies were identified from PubMed, Springer Link, Ovid, Chinese Wanfang Data Knowledge Service Platf...

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Current understanding in diagnosis and management of factor XIII deficiency

Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resis...

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Advances of Coagulation Factor XIII

OBJECTIVE To provide a comprehensive literature review on roles of coagulation factor XIII (FXIII) in coagulation, wound healing, neoplasm, bone metabolism, and pregnancy. DATA SOURCES All articles in PubMed with key words "Coagulation factor XIII", "wound", "leukemia", "tumor", "bone," and "pregnancy" with published date from 2001 to 2016 were included in the study. Frequently cited publicat...

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Factor XIII deficiency.

Inherited factor XIII (FXIII) deficiency is a rare bleeding disorder that can present with umbilical bleeding during the neonatal period, delayed soft tissue bruising, mucosal bleeding and life-threatening intracranial haemorrhage. FXIII deficiency has also been associated with poor wound healing and recurrent miscarriages. FXIII plays an integral role in haemostasis by catalysing the cross-lin...

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ژورنال

عنوان ژورنال: British Journal of Haematology

سال: 2008

ISSN: 0007-1048

DOI: 10.1111/j.1365-2141.1991.tb08629.x