2012 Fiscal Year Final Research Report
Separation of VWF domain function using gene-targeted mic
| Project/Area Number |
22591059
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Hematology
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| Research Institution | Nagoya University |
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Principal Investigator |
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| Project Period (FY) |
2010 – 2012
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| Keywords | 血友病 / von Willebrand病 / von Willebrand因子 / 第VIII因子 / ADAMTS13 |
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| Research Abstract |
von Willebrand factor is a multi-functional hemostatic factor that plays an important role of primary hemostasis. The VWF multi-function is accomplished by its multi-domain structure and each functional module may contribute to different hemostatic functions. We crossed VWFK1362A knock in mouse of which Lys1362, an important GPIb binding site, is mutated to Ala and VWF-/- mouse to yield the in vivo model of quantitative variations of functional/nonfunctional VWF models. We also introduced CAST/Ei background to increase the basic plasma VWF levels. VWFK1362A showed severe bleeding diathesis, suggesting VWF GPIb binding is pivotal function to maintain hemostasis. Crossing between VWFK1362A and VWF-/- mouse, however, was partially successful, because of infertility and prey. Further investigation would reveal quantitative function of VWF to maintain hemostasis in mammals.
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Research Products
(34 results)
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[Presentation] 名古屋大学における血友病Bの血液凝固第IX 因子遺伝子解析2012
Author(s)
村田 萌, 奥山 恵理子, 鈴木 敦夫, 宮脇 由理, 高木明, 村手 隆, 松下 正, 鈴木 伸明, 林 磨由子, 齋藤 英彦, 小嶋 哲人
Organizer
第34回日本血栓止血学会学術集会
Place of Presentation
東京
Year and Date
2012-06-08
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[Presentation] 複合的遺伝子再構成を認めた重症血友病A症例2010
Author(s)
宮脇由理, 鈴木敦夫, 藤森祐多, 山田貴之, 高木明, 村手隆, 鈴木伸明, 勝美章, 山本晃士, 松下正, 小嶋哲人
Organizer
第33回日本血栓止血学会学術集会
Place of Presentation
鹿児島
Year and Date
2010-04-23
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