Role of blood coagulation factor XIII in vascular diseases.
نویسنده
چکیده
FXIII, also called fibrin-stabilising factor [1] , is a transglutaminase composed of two A subunits and two B subunits circulating in plasma as a tetramer (A2B2) (Figure 1). FXIII plays an important role in clot stabilisation by cross-linking fibrin chains [2] after activation by thrombin (Figure 1). In addition, a number of other proteins are substrates for activated FXIII, such as α2-antiplasmin, von Willebrand factor, factor V, thrombospondin, vitronection and thrombin-activable fibrinolysis inhibitor (TAFI) [3–7]. All A-subunit molecules in plasma are in complex with the B-subunit (carrier protein) at a concentration of approximately 21 μg/ml, whereas the B-subunit is present in both free and complexed form [8]. FXIII was discovered over 70 years ago by Barkan et al., who observed the insolubility of fibrin clots in the presence of calcium [9]. Until recently little was known about the role of FXIII in vascular diseases, since most studies have been carried out in patients with congenital FXIII deficiency, which results in a serious bleeding diathesis and defective wound healing [10]. These patients show a complete absence of the A-subunit in plasma due to different mutations in the A-subunit gene [11]. 31 Minireview S W I S S M E D W K LY 2 0 0 1 ; 1 3 1 : 3 1 – 3 4 · w w w. s m w. c h
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عنوان ژورنال:
- Swiss medical weekly
دوره 131 3-4 شماره
صفحات -
تاریخ انتشار 2001