A véralvadás XIII-as faktora = Blood coagulation factor XIII.

I. A XIII-as faktor (FXIII) strukturaja es funkcioja Az aktivacios peptid nelkul a FXIII-A instabil. A FXIII aktivaciot a plazmaban a fibrin polimerizacio determinalja, a FXIII-A Val34Leu polimorfizmusnak csak modulalo hatasa van. A FXIII A es B alegysegenek kapcsolodasat gatlo monoklonalis antitestek előallitasa. A cellularis FXIII szerepet jatszik a monocytak/macrophagok phagocytosisaban. Az alvadekban aktivalodo granulocytakbol felszabadult proteazok lebontjak a FXIIIa-t. II. Klinikai FXIII kutatasok Uj tipusu modszer a FXIII (ill. mas transzglutaminazok) meresere, ill. a FXIII-A Val34Leu polimorfizmus kimutatasara. Az intracellularis FXIII-A aramlasos citometrias kimutatasara uj modszer, mely kivaloan alkalmazhato az acut myleoid leukemiak diagnosztikajaban. Csontvelő ablacio soran szignifikansan csokken a plazma FXIII szintje, magas thrombocyta szammal jaro myeloproliferativ megbetegedesekben viszont emelkedik. Nőkben az emelkediett FXIII szint tobb mint ketszeresere noveli a myocardialis infarctus rizikojat nőkben. Kronikus bronchoalveolaris megbetegedesekben jelentősen emelkedik a bronchoalveolaris mosofolyadekban a FXIII mennyisege. 4 uj mutaciot irtunk le FXIII hianyos betegekben, s analizaltuk ezek feherje biokemiai kovetkezmenyeit. FXIII hianyios transzgen egereken igazoltuk, hogy a FXIII szukseges a normalis sebgyogyulashoz. | I. Structure and function of factor XIII (FXIII) The absence of activation peptide makes FXIII-A instable. The activation of FXIII in the plasma is determined by fibrin polymerization; FXIII-A Val34Leu polymorphism only modulates activation. Production of monoclonal antibodies with inhibitory effect on the association of FXIII A and B subunits. Cellular FXIII plays a role in the phagocytosis by monocytes/macrophages. Proteases released from granulocytes in the clot break down activated FXIII. Clinical studies on FXIII New methods on the measurement of FXIII activity, and on the detection of FXIII-A Val34Leu polymorphism. Method on the detection of intracellular FXIII-A and its application to the diagnosis of acute myeloid leukemias. Bone marrow ablation results in the significant decrease of plasma FXIII level; in myeloproliferative diseases with high platelet count plasma FXIII is elevated. In women elevated FXIII level increases the risk for myocardial infarction by more than two-folds. In chronic bronchoalveolar inflammation the amount of FXIII in the lavage fluid is significantly increased. Description of four new mutations in FXIII deficient patients, and protein structural analysis of their consequences. It was demonstrated on FXIII deficient transgene mice that FXIII is required for normal wound healing.