Document Detail

Male-specific cardiac pathologies in mice lacking either the A or B subunit of factor XIII.
MedLine Citation:
PMID:  18278192     Owner:  NLM     Status:  MEDLINE    
Factor XIII (FXIII) is a proenzyme of plasma transglutaminase consisting of enzymatic A subunits (FXIII-A) and non-catalytic B subunits (FXIII-B), and acts in haemostasis and wound healing. We generated mice lacking either FXIII-A or FXIII-B to investigate the physiological functions of FXIII in vivo. A longitudinal study was carried out using the gene-targeted mice to explore the possible effects of FXIII deficiency on aging. Survival rates of FXIII-A(-/-) males decreased to approximately 50% at 10 months after birth, although most FXIII-A(-/-) females and both genders of wild-type mice survived. Four FXIII-A(-/-) males died of severe intra-thoracic haemorrhage, and a large haematoma was found in their hearts. Haemorrhage, haemosiderin deposition and/or fibrosis were observed in the hearts of other dead FXIII-A(-/-) males. Fibrosis together with haemosiderin deposition was also found in the hearts of FXIII-A(-/-) males sacrificed. The in-vivo cardiac function was normal in FXIII-A(-/-) mice when compared with wild-type mice despite the presence of significant cardiac fibrosis. Although survival rates for both genders of the FXIII-B(-/-) and wild-type mice did not differ, mild fibrosis together with haemosiderin deposits were only found in the hearts of the sacrificed FXIII-B(-/-) males. Carditis and fibrosis in FXIII-deficient mice might be caused by a faulty or delayed reparative process that was initiated by abnormal haemorrhagic events within heart tissue. It is important therefore to examine possible cardiac involvement in human patients with congenital FXIII deficiency.
Masayoshi Souri; Shiori Koseki-Kuno; Naoki Takeda; Mitsunori Yamakawa; Yasuchika Takeishi; Jay L Degen; Akitada Ichinose
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Thrombosis and haemostasis     Volume:  99     ISSN:  0340-6245     ISO Abbreviation:  Thromb. Haemost.     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-02-18     Completed Date:  2008-04-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7608063     Medline TA:  Thromb Haemost     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  401-8     Citation Subset:  IM    
Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University, School of Medicine, Iida-Nishi 2-2-2, Yamagata 990-9585, Japan.
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MeSH Terms
Age Factors
Aging / blood,  pathology
Factor XIII / genetics,  metabolism*
Factor XIII Deficiency / blood,  complications*,  genetics,  pathology
GTP-Binding Proteins / metabolism
Heart Diseases / blood,  etiology,  genetics,  pathology*
Hematoma / blood,  etiology,  pathology
Hemorrhage / blood,  etiology,  pathology
Hemosiderin / metabolism
Mice, Inbred C57BL
Mice, Knockout
Myocarditis / blood,  etiology,  pathology
Myocardium / enzymology,  metabolism,  pathology*
Sex Factors
Transglutaminases / metabolism
Grant Support
Reg. No./Substance:
104641-95-4/factor XIII subunit A; 9011-92-1/Hemosiderin; 9013-56-3/Factor XIII; 97089-56-0/factor XIIIb; EC 2.3.2.-/transglutaminase 2; EC; EC 3.6.1.-/GTP-Binding Proteins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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