Factor XIII, an enzyme that cross-links fibrin, belongs to the blood coagulation system. [ 1, 2] Screening for factor XIII, also known as fibrin Factor XIII, also referred to as fibrin stabilizing factor, plays a crucial role in the coagulation cascade by enhancing the stability of blood clot formation. Nugent DJ. Prophylaxis in rare coagulation disorders -- factor XIII deficiency. Thromb Res. ; Suppl 1:S Anwar R, Gallivan L, Richards M, Khair K, Wright M, Minford A. Factor XIII deficiency: new nonsense and deletion mutations in the human factor XIIIA gene. Haematologica. Dec;90(12) 15 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 Factor XIII (FXIII) deficiency is a rare congenital bleeding disorder estimated to affect 1 in 2 million live births. Treatment often involves prophylaxis with FXIII concentrate and is especially important in preventing intracranial hemorrhage (ICH) and maintaining pregnancy in women of childbearing age. The rarity of this condition and lack of The activation and regulation of coagulation Factor XIII (FXIII) protein has been the subject of active research for the past three decades. Although discrete evidence exists on various aspects of FXIII activation and regulation a combinatorial structure/functional view in this regard is lacking. In D-dimer is a terminal degradation product from the breakdown of fibrin. Unlike other fibrin degradation products, D-dimer is formed only after fibrin has been cross-linked by activated factor XIII and lysed by plasmin [ 78 ]. Quantitative D-dimer is most often used in the evaluation of venous thrombosis and DIC
Coagulation factor XIII activity predicts left ventricular …
Introduction. Congenital factor XIII (FXIII) deficiency is chiefly caused by mutations in the F13A gene (95% of cases) and, more rarely, by F13B gene defects (5% of cases). The F13A gene, coding for the FXIII A protein subunit, occupies chromosomal position 6p24–25 and comprises 15 exons encoding a amino acid protein. 1 AP-FXIII: Activation peptide factor XIII; FXIII: Factor XIII; FXIIIa: Activated factor XIII; Gln: Glutamine; Lys: Lysine. As a TG, FXIII catalyzes the Coagulation factor XIII (FXIII) is a transglutaminase with a well defined role in the final stages of blood coagulation. Active FXIII (FXIIIa) catalyzes the formation of ϵ- (γ-glutamyl)lysine isopeptide bonds between specific Gln and Lys residues. The primary physiological outcome of this catalytic activity is stabilization of the fibrin clot Abstract. 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 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,
Coagulation factor XIII is a critical driver of liver regeneration ...
Methods: Venous blood was withdrawn from 12 volunteers in a crossover study. Four percent succinylated gelatin was added to citrated whole-blood samples to make a 40 vol% end-concentration of gelatin. The baseline and 40 vol% samples, and samples with addition of fresh-frozen plasma (FFP), fibrinogen, coagulation factors XIII (FXIII) or VIII Introduction. Coagulation factor XIII is a protransglutaminase that has a major role in the final stage of blood coagulation [].The zymogen plasma FXIII (pFXIII) circulates in plasma as a tetramer (FXIII-A 2 B 2) consisting of two catalytic A subunits (FXIII-A) and two carrier/protective B subunits (FXIII-B).FXIII-A is produced by cells of bone Abstract. Coagulation factor XIII (FXIII) is converted by thrombin into its active form, FXIIIa, which crosslinks fibrin fibers, rendering clots more stable and resistant to degradation. FXIII affects fibrin clot structure and function leading to a more prothrombotic phenotype with denser networks, characterizing patients at risk of venous Factor XIII is activated by thrombin and calcium ion to a transglutaminase that catalyzes the formation of gamma-glutamyl-epsilon-lysine cross-links between fibrin chains, thus stabilizing the fibrin clot. Also cross-link alphaplasmin inhibitor, or fibronectin, to the alpha chains of fibrin. 1 publication PG, et al. Coagulation factor XIII A and B subunits in bone marrow and liver transplantation. Transplantation. ; 43 (1): Targeted inactivation of the mouse locus encoding coagulation factor XIII-A: hemostatic abnormalities in mutant mice and characterization of the coagulation deficit. Thromb Haemost. ; 88 (6) Coagulation factor XIII serves as protein disulfide isomerase. Thrombosis and haemostasis , – (). Article CAS Google Scholar