................................................................................................................................. I ACKNOWLEDGEMENTS ............................................................................................................ III PUBLICATIONS ARISING FROM THIS THESIS .............................................................................. V TABLE OF CONTENT...

XIII

For the assay of blood-clotting Factor XIII (Fibrin-Stabilizing Factor, FSF), the antibody-neutralization test (Bohn and Haupt, 1968), the monodancylcadaverine incorporation test (Lorand et al., 1969; Nishimura et al., 1975) and the Laurell method are tried in our country (Hidano et al., 1975). None of them, however, could be applied to the clinical screening for Factor XIII-deficient cases; th...

A congenital deficiency of the coagulation Factor XIII A subunit (F XIII A) is a rare autosomal recessive disorder that is characterized by a life-long bleeding tendency complicated by a difficulty in healing. Thus far, no molecular genetic analysis of this disorder has been reported. In this study, we demonstrate the molecular abnormalities in a family with this disorder. We performed Northern...

The Park Place Economist, Volume XIII

....................................................................................................................... I PREFACE......................................................................................................................... III LIST OF PUBLICATIONS...............................................................................................VII LIST OF FIGURES ..........

..... ............................................................................................................... X Acknowledgements ...................................................................................................XII Declaration... .......................................................................................................... XIII Chapter

Factor XIII is the fibrin-stabilizing factor that covalently cross-links fibrin monomers to form a highly organized, stable fibrin clot. The plasma form of factor XIII is a heterodimer, a2b2, consisting of two a-chains and two b-chains; the intracellular form, such as in platelets and placenta, is a dimer, a2, consisting of a-chains only. The catalytic function of factor XIII, a transglutaminas...

Transglutaminases are a family of enzymes that catalyze the formation of epsilon-(gamma-glutamyl)lysine isopeptide bonds in proteins, an activity that has been implicated in the pathogenesis of cartilage matrix mineralization in degenerative arthritis. Type II transglutaminase and thrombin-activatable factor XIII have been identified in articular cartilage. Thrombin, a coagulation protease, is ...