• Search Research Projects
  • Search Researchers
  • How to Use
  1. Back to previous page

Separation of VWF domain function using gene-targeted mic

Research Project

Project/Area Number 22591059
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Hematology
Research InstitutionNagoya University

Principal Investigator

MATSUSHITA Tadashi  名古屋大学, 医学部附属病院, 教授 (30314008)

Project Period (FY) 2010 – 2012
Project Status Completed (Fiscal Year 2012)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2012: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2011: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2010: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Keywords血友病 / von Willebrand病 / von Willebrand因子 / 第VIII因子 / ADAMTS13 / von willebrand因子
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.

Report

(4 results)
  • 2012 Annual Research Report   Final Research Report ( PDF )
  • 2011 Annual Research Report
  • 2010 Annual Research Report
  • Research Products

    (52 results)

All 2013 2012 2011 2010

All Journal Article (19 results) (of which Peer Reviewed: 18 results) Presentation (15 results) Book (18 results)

  • [Journal Article] Increased von Willebrand Factor to ADAMTS13 ratio as a predictor of thrombotic complications following a major hepatectomy2012

    • Author(s)
      Kobayashi S , Matsushita T
    • Journal Title

      Arch Surg

      Volume: 147(10) Issue: 10 Pages: 909-17

    • DOI

      10.1001/archsurg.2012.998

    • Related Report
      2012 Annual Research Report 2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] A possible mechanismfor Inv22-related F8 large deletions in severe hemophilia A patients with high responding factor VIII inhibitors2012

    • Author(s)
      J. Fujita, Y. Miyawaki, A. Suzuki, et al.
    • Journal Title

      Journal of Thrombosis and Haemostasis

      Volume: 10 Issue: 10 Pages: 2099-2107

    • DOI

      10.1111/j.1538-7836.2012.04897.x

    • Related Report
      2012 Annual Research Report 2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Thrombosis from a prothrombin mutation conveying antithrombin resistance.2012

    • Author(s)
      Miyawaki Y, Suzuki A, Fujita J, Maki A, Okuyama E, Murata M, Takagi A, Murate T, Kunishima S, Sakai M, Okamoto K, Matsushita T, Naoe T, Saito H, Kojima T
    • Journal Title

      N Engl J Med.

      Volume: 366 Issue: 25 Pages: 2390-2396

    • DOI

      10.1056/nejmoa1201994

    • Related Report
      2012 Annual Research Report 2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Clinical pharmacological study of a plasma-derived factor VIIa and factor X mixture in haemophilia patients with inhibitors - Phase I trial2012

    • Author(s)
      Shirahata A, Matsushita T
    • Journal Title

      Haemophilia.

      Volume: 18(1) Issue: 1 Pages: 94-101

    • DOI

      10.1111/j.1365-2516.2011.02548.x

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] A novel ENG mutation causing impaired co-translational processing of endoglin associated with hereditary hemorrhagic telangiestasia2012

    • Author(s)
      Atsuo Suzuki
    • Journal Title

      Thrombosis Research

      Volume: (in press) Issue: 5 Pages: e200-e208

    • DOI

      10.1016/j.thromres.2011.12.030

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Stress Augments Insulin Resistance and Prothrombotic State : Role of Visceral Adipose-Derived Monocyte Chemoattractant Protein-12012

    • Author(s)
      Uchida Y
    • Journal Title

      Diabetes

      Volume: (In press) Issue: 6 Pages: 1552-61

    • DOI

      10.2337/db11-0828

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Life-threatening hemorrhage and prolonged wound healing are remarkable phenotypes manifested by complete plasminogen activator inhibitor-1 deficiency in humans2011

    • Author(s)
      T Iwaki, A Tanaka, Y Miyawaki, A Suzuki, T Kobayashi, J Takamatsu, T Matsushita, K Umemura, T Urano, T Kojima, T Terao, N Kanayama
    • Journal Title

      J Thromb Haemost

      Volume: 9(6) Issue: 6 Pages: 1200-1206

    • DOI

      10.1111/j.1538-7836.2011.04288.x

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Successful fertility management of a patient with factor V deficiency: planned transfusion of fresh frozen plasma under infertility treatment2011

    • Author(s)
      Iwase A, Matsushita T
    • Journal Title

      Fertil Steril

      Volume: 95(6) Issue: 6 Pages: 2124.e5-2124.e7

    • DOI

      10.1016/j.fertnstert.2011.01.011

    • Related Report
      2012 Final Research Report 2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Pitavastatin-induced angiogenesis and arteriogenesis is mediated by Notchl in a murine hindlimb ischemia model without induction of VEGF2011

    • Author(s)
      Kikuchi R
    • Journal Title

      Lab Invest

      Volume: 91 Issue: 5 Pages: 691-703

    • DOI

      10.1038/labinvest.2011.5

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Virus-associated hemophagocytic syndrome caused by pandemic swine-origin influenza A (H1N1) in a patient after unrelated bone marrow transplantation2011

    • Author(s)
      Katsumi A
    • Journal Title

      J Clin Exp Hematop

      Volume: 51 Pages: 63-65

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Successful fertility management of a patient with factor Vdeficiency : planned transfusion of fresh frozen plasma under infertility treatment.2011

    • Author(s)
      Iwase A
    • Journal Title

      Fertil Steril

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Severe hemophilia A in a Japanese female caused by an F8-intron 22 inversion associated with skewed X chromosome inactivation2010

    • Author(s)
      Miyawaki Y, Matsushita T
    • Journal Title

      Int J Hematol

      Volume: 92(2) Issue: 2 Pages: 405-8

    • DOI

      10.1007/s12185-010-0659-9

    • NAID

      10026712691

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Heterozygous antithrombin deficiency improves in vivo haemostasis in factor VIII-deficient mice2010

    • Author(s)
      Bolliger D, Matsushita T
    • Journal Title

      Thromb Haemost

      Volume: 103(6) Issue: 06 Pages: 1233-8

    • DOI

      10.1160/th09-10-0732

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] A novel splice site mutation inintron C of PROS1 leads to markedly reduced mutant mRNA level, absence ofthrombin-sensitive region, and impaired secretion and cofactor activity of mutant protein S2010

    • Author(s)
      Okada H, Matsushita T
    • Journal Title

      Thromb Res

      Volume: 125(5) Issue: 5 Pages: e246-e250

    • DOI

      10.1016/j.thromres.2009.11.029

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Impaired secretion of carboxyl-terminal truncated factor VII due to an F7 nonsense mutation associated with FVII deficiency2010

    • Author(s)
      Tanaka R, Matsushita T
    • Journal Title

      Thromb Res

      Volume: 125(3) Issue: 3 Pages: 262-6

    • DOI

      10.1016/j.thromres.2009.09.014

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Heterozygous antithrombin deficiency improves in vivo haemostasis in factor VIII-deficient mice.2010

    • Author(s)
      Bolliger D.
    • Journal Title

      Thromb Haemost

      Volume: 103 Pages: 1233-1238

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Severe hemophilia A in a Japanese female caused by an F8-intron 22 inversion associated with skewed X chromosome inactivation.2010

    • Author(s)
      Miyawaki Y.
    • Journal Title

      Int J Hematol

      Volume: 92 Pages: 405-408

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Impaired secretion of carboxyl-terminal truncated factor VII due to an F7 nonsense mutation associated with FVII deficiency.2010

    • Author(s)
      Tanaka R.
    • Journal Title

      Thromb Res

      Volume: 125 Pages: 262-266

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] 消費性凝固傷害2010

    • Author(s)
      松下正
    • Journal Title

      徹底ガイドDICのすべて-基礎と診療の最前線-救急・集中治療

      Volume: 22 Pages: 1420-1428

    • Related Report
      2010 Annual Research Report
  • [Presentation] 静脈血栓塞栓症リスク・アンチトロンビン抵抗性とそのスクリーニング検査法2012

    • Author(s)
      高木明, 宮脇 由理, 鈴木 敦夫, 藤田 絢子, 牧明日加,奥山 恵理子, 村田 萌, 村手 隆, 松下 正, 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
    • Related Report
      2012 Final Research Report
  • [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
    • Related Report
      2012 Final Research Report
  • [Presentation] 抗VWFインヒビター保有von Willebrand Disease(VWD)Type3症例における製剤選択2012

    • Author(s)
      鈴木 伸明,三田 直美, 松下 正, 小嶋 哲人, 山本 晃士, 勝見 章,林 磨由子, 梶浦 容子, 高津 真由美, 直江 知樹
    • Organizer
      第60回日本輸血・細胞治療学会総会
    • Place of Presentation
      福島
    • Year and Date
      2012-05-25
    • Related Report
      2012 Final Research Report
  • [Presentation] 静脈血栓塞栓症リスク・アンチトロンビン抵抗性とそのスクリーニング検査法2012

    • Author(s)
      高木 明
    • Organizer
      第34回日本血栓止血学会学術集会
    • Place of Presentation
      ハイアットリージェンシー東京
    • Related Report
      2012 Annual Research Report
  • [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
    • Organizer
      第34回日本血栓止血学会学術集会
    • Place of Presentation
      ハイアットリージェンシー東京
    • Related Report
      2012 Annual Research Report
  • [Presentation] .抗VWFインヒビター保有von Willebrand Disease(VWD)Type3症例における製剤選択.2012

    • Author(s)
      鈴木 伸明
    • Organizer
      第60回日本輸血・細胞治療学会総会
    • Place of Presentation
      ホテルハマツ
    • Related Report
      2012 Annual Research Report
  • [Presentation] 凝固傷害に対する補充療法2011

    • Author(s)
      松下正
    • Organizer
      第18回日本輸血・細胞治療学会秋季シンポジウム
    • Place of Presentation
      埼玉
    • Year and Date
      2011-10-21
    • Related Report
      2012 Final Research Report
  • [Presentation] 凝固傷害に対する補充療法2011

    • Author(s)
      松下正
    • Organizer
      第18回日本輸血・細胞治療学会秋季シンポジウム
    • Place of Presentation
      埼玉県県民健康センター
    • Year and Date
      2011-10-21
    • Related Report
      2011 Annual Research Report
  • [Presentation] R702C Mutation of the MYH9 Gene causes great changes in blood cell and other organs in mice model2011

    • Author(s)
      Nobuaki Suzuki
    • Organizer
      第73回日本血液学会学術集会
    • Place of Presentation
      名古屋国際会議場
    • Year and Date
      2011-10-15
    • Related Report
      2011 Annual Research Report
  • [Presentation] 重症血友病A3症例に見られた血液凝固第VIII因子遺伝子イントロン22逆位を伴う特異なX染色体再構成2011

    • Author(s)
      藤田絢子,牧明日加,奥山恵理子,村田萌,松下正 他
    • Organizer
      第73回日本血液学会学術集会
    • Place of Presentation
      名古屋
    • Year and Date
      2011-10-14
    • Related Report
      2012 Final Research Report
  • [Presentation] GPIIb 遺伝子において複合へテロ変異を認めた血小板無力症の一症例解析2011

    • Author(s)
      藤田 絢子,牧 明日加,奥山恵理子,村田 萌,宮脇 由理,鈴木 敦夫,松下 正、 國島 伸治,高木 明,小嶋哲人
    • Organizer
      第12回日本検査血液学会学術集会
    • Place of Presentation
      倉敷
    • Year and Date
      2011-07-18
    • Related Report
      2012 Final Research Report
  • [Presentation] アンチトロンビン抵抗性を検出するトロンビン不活化動態解析法の開発2011

    • Author(s)
      高木 明,宮脇由理,鈴木 敦夫,藤田 絢子,牧 明日加,奥山恵理子,村田 萌,村手 隆,松下 正,小嶋 哲人
    • Organizer
      第12回日本検査血液学会学術集会
    • Place of Presentation
      倉敷
    • Year and Date
      2011-07-18
    • Related Report
      2012 Final Research Report
  • [Presentation] A case of type 3 von Willebrand Disease who developed anaphylactic anti-VWF inhibitor2010

    • Author(s)
      Nobuaki Suzuki, Naomi Sanda, Tadashi Matsushita, Tetsuhito Kojima, Koji Yamamoto, Akira Katsumi, Kanji Hirashima, Yoko Kajiura, Mayumi Takatsu, Tomoki Naoe
    • Organizer
      第72回日本血液学会学術集会
    • Place of Presentation
      横浜
    • Year and Date
      2010-09-25
    • Related Report
      2012 Final Research Report
  • [Presentation] 新規複合ヘテロ変異による先天性第V因子欠乏症の一症例2010

    • Author(s)
      鈴木敦夫, 宮脇由理, 藤森祐多, 高木明, 村手隆, 山本晃士, 松下正, 高松純樹, 小嶋哲人
    • Organizer
      第11回日本検査血液学会学術集会
    • Place of Presentation
      東京
    • Year and Date
      2010-07-25
    • Related Report
      2012 Final Research Report
  • [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
    • Related Report
      2012 Final Research Report
  • [Book] Annual Review 血液 20132013

    • Author(s)
      松下正 鈴木伸明
    • Total Pages
      247
    • Publisher
      中外医学社
    • Related Report
      2012 Annual Research Report
  • [Book] 【知っておきたい内科症候群】 血液《血小板・凝固系の異常》 播種性血管内凝固症候群、内科(0022-1961)2012

    • Author(s)
      松下正
    • Related Report
      2012 Final Research Report
  • [Book] 臨床雑誌 内科 2012年109巻6号2012

    • Author(s)
      松下正
    • Total Pages
      1516
    • Publisher
      南江堂
    • Related Report
      2012 Annual Research Report
  • [Book] 消化器外科 2012年第35巻第5号2012

    • Author(s)
      松下正
    • Total Pages
      189
    • Publisher
      へるす出版
    • Related Report
      2012 Annual Research Report
  • [Book] 【一般内科医がみる血液疾患 血液専門医との効率的な連携のために】一般内科医が対応できる血液疾患の治療 【薬剤使用時の注意事項】処置・手術時の抗凝固薬、抗血小板薬の扱い、Medicina2011

    • Author(s)
      松下正
    • Related Report
      2012 Final Research Report
  • [Book] 症状からアプローチするプライマリケア】出血傾向(紫斑・点状出血)2011

    • Author(s)
      松下正
    • Publisher
      日本医師会雑誌
    • Related Report
      2012 Final Research Report
  • [Book] 日本血栓止血学会後天性血友病A診療ガイドライン2011

    • Author(s)
      田中一郎,天野景裕,松下正,日笠聡,嶋緑倫,瀧正志,他
    • Publisher
      日本血栓止血学会誌
    • Related Report
      2012 Final Research Report
  • [Book] 白血病診療ポケットブック2011

    • Author(s)
      松下正
    • Publisher
      中外医学社
    • Related Report
      2012 Final Research Report
  • [Book] 【DIC診断・治療の最前線】DICの治療 最近のトピックス アンチトロンビン濃縮製剤とへパリン・ヘパリン類似物質、医学の歩み2011

    • Author(s)
      松下正
    • Related Report
      2012 Final Research Report
  • [Book] 【抗血栓両方の新潮流-作用機序に基づく治療戦略】抗血栓療法の進歩 静脈血栓症における一次予防と二次予防、カレントテラピー2011

    • Author(s)
      松下正
    • Related Report
      2012 Final Research Report
  • [Book] 身近な話題・世界の話題 重度出血を有する外傷患者におけるトラネキサム酸の効果(CRASH-2) 無作為化プラセボ比較対照試験2011

    • Author(s)
      松下正
    • Publisher
      血液フロンティア/(株)医薬ジャーナル社
    • Related Report
      2012 Final Research Report
  • [Book] 【徹底ガイド DICのすべて 基礎と診療の最前線】 病態生理と病理 消費性凝固障害2010

    • Author(s)
      松下正
    • Publisher
      救急・集中治療/(株)総合医学社
    • Related Report
      2012 Final Research Report
  • [Book] 【血管合併症の予防・治療をめぐって】 血管合併症の予防・治療をめぐる最新の展開 抗血小板剤・抗凝固療法を中心として2010

    • Author(s)
      太田寛、松下正、宮地茂、土肥博雄、河北誠
    • Publisher
      日本臨床内科医会
    • Related Report
      2012 Final Research Report
  • [Book] 【血管合併症の予防・治療をめぐって】 ワーファリンによる血管合併症の予防・治療におけるマネジメント 検査・処置・手術における取扱い2010

    • Author(s)
      松下正
    • Publisher
      日本臨床内科医会会誌/(一社)日本臨床内科医会
    • Related Report
      2012 Final Research Report
  • [Book] 【癌と血栓症】 臨床 造血器腫瘍と血栓症2010

    • Author(s)
      松下正
    • Publisher
      血栓と循環/(株)メディカルレビュー社
    • Related Report
      2012 Final Research Report
  • [Book] 【血液疾患の診かた 血液専門医以外のための血液疾患対応マニュアル】 プライマリ・ケア医に必要な血液疾患の知識 血友病鈴木伸明2010

    • Author(s)
      松下正
    • Publisher
      治療/ (株)南山堂
    • Related Report
      2012 Final Research Report
  • [Book] 【血栓症】 種々の病態と血栓症:最近の進歩 骨髄増殖性疾患と血栓症2010

    • Author(s)
      松下正
    • Publisher
      最新医学/(株)最新医学社
    • Related Report
      2012 Final Research Report
  • [Book] フォン・ヴィレブランド病診断における臨床検査2010

    • Author(s)
      松下正
    • Total Pages
      41
    • Publisher
      シスメックス株式会社学術本部
    • Related Report
      2010 Annual Research Report

URL: 

Published: 2010-08-23   Modified: 2019-07-29  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi