Factor XIII deficiency enhances thrombin generation due to impaired fibrin polymerization — An effect corrected by Factor XIII replacement
نویسندگان
چکیده
منابع مشابه
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...
متن کامل[Congenital deficiency of fibrin stabilizing factor [factor XIII)].
The authors report a new case of congenital deficiency of fibrin stabilizing factor. The defect is transmitted as an autosomal recessive trait. The earliest symptom is umbilical bleeding when the cord separates. The diagnosis is confirmed by the rapid dispersion of the clot in urea 5 M or monochloroacetic acid.
متن کاملFactor 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...
متن کاملFactor XIIIa Formation Promoted by Complexing of a - Thrombin , Fibrin , and Plasma Factor XIII
Fibrin polymers (des A,B fibrinogen) reduced the concentration of a-thrombin required for 50% activation of plasma factor XIII (a2b2 tetramer) by approximately 1 00-fold. In the presence of fibrin, the amount of ‘y-thrombin required for activation was not affected. Catalytically inactive i-Pr2Pand D-Phe-PrO-Arg-CH2-a-thrOmbin were found to inhibit over 95% of the activation by a-thrombin in the...
متن کاملFibrin cross-linking in congenital factor XIII deficiency.
Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. Quantitative studies of transamidase activity of the factor are available in only a few cases, and the fibrin cross-linking pattern is not well known. The present paper deals with the quantitative estimation of factor XIII transamidase activity (dansylcadaverine ...
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ژورنال
عنوان ژورنال: Thrombosis Research
سال: 2017
ISSN: 0049-3848
DOI: 10.1016/j.thromres.2016.11.012