| Male-specific cardiac pathologies in mice lacking either the A or B subunit of factor XIII. | |
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MedLine Citation:
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PMID: 18278192 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Masayoshi Souri; Shiori Koseki-Kuno; Naoki Takeda; Mitsunori Yamakawa; Yasuchika Takeishi; Jay L Degen; Akitada Ichinose |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Thrombosis and haemostasis Volume: 99 ISSN: 0340-6245 ISO Abbreviation: Thromb. Haemost. Publication Date: 2008 Feb |
Date Detail:
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Created Date: 2008-02-18 Completed Date: 2008-04-22 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7608063 Medline TA: Thromb Haemost Country: Germany |
Other Details:
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Languages: eng Pagination: 401-8 Citation Subset: IM |
Affiliation:
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Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University, School of Medicine, Iida-Nishi 2-2-2, Yamagata 990-9585, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Age Factors Aging / blood, pathology Animals Echocardiography Factor XIII / genetics, metabolism* Factor XIII Deficiency / blood, complications*, genetics, pathology Female Fibrosis GTP-Binding Proteins / metabolism Heart Diseases / blood, etiology, genetics, pathology* Hematoma / blood, etiology, pathology Hemorrhage / blood, etiology, pathology Hemosiderin / metabolism Male Mice Mice, Inbred C57BL Mice, Knockout Myocarditis / blood, etiology, pathology Myocardium / enzymology, metabolism, pathology* Sex Factors Transglutaminases / metabolism |
| Grant Support | |
ID/Acronym/Agency:
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AR 49822/AR/NIAMS NIH HHS; HL 71555/HL/NHLBI NIH HHS |
| Chemical | |
Reg. No./Substance:
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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 2.3.2.13/Transglutaminases; EC 3.6.1.-/GTP-Binding Proteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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