title: Impaired clot retraction in factor XIII A subunit-deficient mice.
abstract: Factor XIII (FXIII) is a plasma transglutaminase that cross-links fibrin monomers, alpha(2)-plasmin inhibitor, and so forth. Congenital FXIII deficiency causes lifelong bleeding symptoms. To understand the molecular pathology of FXIII deficiency in vivo, its knockout mice have been functionally analyzed. Because prolonged bleeding times, a sign of defective/abnormal primary hemostasis, were commonly observed in 2 separate lines of FXIII A subunit (FXIII-A) knockout mice, a possible role or roles of FXIII in platelet-related function was investigated in the present study. Although platelet aggregation induced by adenosine diphosphate or collagen was normal, clot retraction (CR) was lost in the platelet-rich plasma (PRP) of FXIII-A knockout mice. In contrast, there was no CR impairment in the PRP of tissue transglutaminase-knockout mice compared with that of wild-type mice. Furthermore, a transglutaminase inhibitor, cystamine, halted CR in the PRP of wild-type mice. These results indicate that the enzymatic activity of FXIII is necessary for CR, at least in mice.
authors.affiliation: Biomembrane Signaling Project 2, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.
authors.forename: Kohji
authors.initials: K
authors.lastname: Kasahara
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Souri
authors.forename: Mizuho
authors.initials: M
authors.lastname: Kaneda
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Miki
authors.forename: Naomasa
authors.initials: N
authors.lastname: Yamamoto
authors.forename: Akitada
authors.initials: A
authors.lastname: Ichinose
mesh_terms: D000244:Adenosine Diphosphate / Q000378:metabolism; D000818:Animals; D003018:Clot Retraction; D003094:Collagen / Q000378:metabolism; D005177:Factor XIII Deficiency / Q000378:metabolism*; D026122:Factor XIIIa / Q000502:physiology*; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D053657:Platelet-Rich Plasma / Q000378:metabolism; D021122:Protein Subunits; D011503:Transglutaminases / Q000502:physiology
chemical_list: D021122:Protein Subunits; D000244:Adenosine Diphosphate; D003094:Collagen; D026122:Factor XIIIa; D011503:Transglutaminases