Home > Journals > Minerva Biotecnologica > Past Issues > Minerva Biotecnologica 2002 June;14(2) > Minerva Biotecnologica 2002 June;14(2):121-8

CURRENT ISSUE
 

JOURNAL TOOLS

eTOC
To subscribe PROMO
Submit an article
Recommend to your librarian
 

ARTICLE TOOLS

Reprints
Permissions

 

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


PDF


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).

top of page