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REVIEWS 7th INTERNATIONAL CONFERENCE ON TRANSGLUTAMINASES AND PROTEIN CROSSLINKING REACTIONS
Ferrara (Italy), September 14-17, 2002
Minerva Biotecnologica 2002 June;14(2):121-8
Copyright © 2003 EDIZIONI MINERVA MEDICA
language: English
Factor XIII: state of the art
Ichinose A.
Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan
Factor XIII is a zymogenic transglutaminase whose expression is specifically restricted to some haematopoietic cells and to placenta. In these cells it is present as a dimer of inactive A subunits. The protein is also present in plasma as a complex with two B subunits, synthetized and secreted by the liver. The conversion into the active crosslinking enzyme is obtained by limited proteolysis and interaction with calcium ions. Once produced, Factor XIIIa crosslinks fibrin aggregates stabilizing them against mechanical stress and proteolytic degradation, incorporating proteinase inhibitors into the fibrin clot. Genetic deficiencies of Factor XIII, resulting in haemorrhagic diseases, are now known and their pathogenesis is discussed at the light of recent molecular biology and in vivo inactivation (knock-out) experiments. I also detail on the recently demonstrated association between specific Factor XIII polymorphic forms and risk of cardiovascular diseases (myocardial infarction and venous thrombosis).