Journal article
The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant mice
International journal of hematology, Vol.91(1), pp.30-35
01/2010
DOI: 10.1007/s12185-009-0477-0
PMID: 20047094
Abstract
Recently, two independent groups have established ADAMTS13-deficient mice using gene-targeting techniques. In humans, genetic or acquired deficiency in ADAMTS13 leads to a potentially fatal syndrome, thrombotic thrombocytopenic purpura (TTP). Surprisingly, ADAMTS13-deficient mice are viable with no apparent signs of TTP. However, these mouse models indicate that ADAMTS13 down-regulates platelet adhesion and aggregation in vivo, and ADAMTS13 deficiency can provide enhanced thrombus formation at the site of vascular lesions. In addition, ADAMTS13 by cleaving hyperactive ultra-large von Willebrand factor multimers not only down-regulates thrombosis but also inflammation. ADAMTS13-congenic mice that carry a truncated form of ADAMTS13 lacking the C-terminal domains have also been developed. Phenotypes of the congenic mice indicate the physiological significance of the C-terminal domains of ADAMTS13 in down-regulating thrombus growth. The studies mentioned here in different mouse models uncover the in vivo function of ADAMTS13 and strengthened the understanding of the mechanism of systemic disease TTP.
Details
- Title: Subtitle
- The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant mice
- Creators
- Fumiaki Banno - National Cardiovascular Center Research Institute, 5-7-1 Fujishirodai, Suita, Osaka, 565-8565, Japan. banno@ri.ncvc.go.jpAnil K ChauhanToshiyuki Miyata
- Resource Type
- Journal article
- Publication Details
- International journal of hematology, Vol.91(1), pp.30-35
- DOI
- 10.1007/s12185-009-0477-0
- PMID
- 20047094
- ISSN
- 0925-5710
- eISSN
- 1865-3774
- Language
- English
- Date published
- 01/2010
- Academic Unit
- Hematology, Oncology, and Blood & Marrow Transplantation; Internal Medicine
- Record Identifier
- 9984094645902771
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