Quality control of secretory proteins containing proline residues in the cis form

• Project/Area Number: 25440060
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Functional biochemistry
• Research Institution: National Cardiovascular Center Research Institute
• Principal Investigator: KOKAME KOICHI 国立研究開発法人国立循環器病研究センター, 研究所, 部長 (40270730)
• Co-Investigator(Renkei-kenkyūsha): HIGUCHI YUKA 国立研究開発法人国立循環器病研究センター, 研究所, 非常勤研究員 (30443477)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000); Fiscal Year 2015: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000); Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2013: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
• Keywords: タンパク質品質管理 / 小胞体 / プロリン異性化酵素 / ADAMTS13

Outline of Final Research Achievements

Catalysis of cis-trans isomerization of proline residues is often necessary for proper protein folding and function. This study was aimed at understanding the functional importance of the proline isomerization in the quality control of secretory proteins. We used MDTCS domains of ADAMTS13 that contain four proline residues in the cis form as a model protein. As the results using cultured cells and mice, it was revealed that the proline isomerization catalyzed by cyclophilin B in the endoplasmic reticulum was important for secretion of the MDTCS domains. At least a subset of cyclophilin B molecules were associated with the protein complexes involved in endoplasmic reticulum-associated degradation.

Research Products

• [Journal Article] Genetic variations in complement factors in patients with congenital thrombotic thrombocytopenic purpura with renal insufficiency. (2016)
  • Author(s): Fan X, Kremer Hovinga JA, Shirotani-Ikejima H, Eura Y, Hirai H, Honda S, Kokame K, Taleghani MM, von Krogh AS, Yoshida Y, Fujimura Y, Lammle B, Miyata T
  • Journal Title: Int J Hematol Volume: 103 Issue: 3 Pages: 283-291
  • DOI: 10.1007/s12185-015-1933-7
  • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
• [Journal Article] Deletion of Herpud1 Enhances Heme Oxygenase-1 Expression in a Mouse Model of Parkinson's Disease (2016)
  • Author(s): Le TM, Hashida K, Ta HM, Takarada-Iemata M, Kokame K, Kitao Y, Hori O
  • Journal Title: Parkinsons Dis. Volume: 1 Pages: 1-9
  • DOI: 10.1155/2016/6163934
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Influence of a rotational speed modulation system used with an implantable continuous-flow left ventricular assist device (EVAHEART) on von Willebrand factor dynamics (2016)
  • Author(s): Noritsugu Naito, Toshihide Mizuno, Takashi Nishimura, Satoru Kishimoto, Yoshiaki Takewa, Yuka Eura, Koichi Kokame, Toshiyuki Miyata, Kazuma Date, Akihide Umeki, Masahiko Ando, Minoru Ono, and Eisuke Tatsumi
  • Journal Title: Artif. Organs Volume: 未定 Issue: 9 Pages: 877-883
  • DOI: 10.1111/aor.12666
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] No association between dysplasminogenemia with p.Ala620Thr mutation and atypical hemolytic uremic syndrome (2016)
  • Author(s): Toshiyuki Miyata, Yumiko Uchida, Yoko Yoshida, Hideki Kato, Masanori Matsumoto, Koichi Kokame, Yoshihiro Fujimura, and Masaomi Nangaku
  • Journal Title: Int. J. Hematol. Volume: 未定
  • Acknowledgement Compliant
• [Journal Article] 先天性TTP（血栓性血小板減少性紫斑病）の遺伝子解析 (2016)
  • Author(s): 樋口（江浦）由佳, 小亀浩市
  • Journal Title: 血栓と循環 Volume: 24
• [Journal Article] 循環器疾患随伴後天性フォンウィルブランド症候群の臨床的インパクト (2016)
  • Author(s): 堀内久徳, 松本雅則, 小亀浩市
  • Journal Title: 日本血栓止血学会誌 Volume: 未定
  • NAID: 130005157425
  • Peer Reviewed
• [Journal Article] ELISA-based detection system for protein S K196E mutation, a genetic risk factor for venous thromboembolism (2015)
  • Author(s): Maruyama K, Akiyama M, Kokame K, Sekiya A, Morishita E, Miyata T
  • Journal Title: PLoS One Volume: 10 Issue: 7 Pages: e0133196-e0133196
  • DOI: 10.1371/journal.pone.0133196
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Exacerbated venous thromboembolism in mice carrying protein S K196E mutation (2015)
  • Author(s): Fumiaki Banno, Toshiyuki Kita, Jose A. Fernandez, Hiroji Yanamoto, Yuko Tashima, Koichi Kokame, John H. Griffin, and Toshiyuki Miyata
  • Journal Title: Blood Volume: 126 Issue: 19 Pages: 2247-2253
  • DOI: 10.1182/blood-2015-06-653162
  • Peer Reviewed / Int'l Joint Research / Acknowledgement Compliant
• [Journal Article] Ndrg1 is a T cell clonal anergy factor negatively regulated by CD28-costimulation and interleukin-2 (2015)
  • Author(s): Yu Mi Oh, Hyung Bae Park, Jae Hun Shin, Ji Eun Lee, Ha Young Park, Dhong Hyo Kho, Jun Sung Lee, Heonsik Choi, Tomohiko Okuda, Koichi Kokame, Toshiyuki Miyata, In-Hoo Kim, Seung Hoon Lee, Ronald Schwartz, and Kyungho Choi
  • Journal Title: Nat. Commun. Volume: 6 Issue: 1 Pages: 8698-8698
  • DOI: 10.1038/ncomms9698
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] 血栓性血小板減少性紫斑病 (2015)
  • Author(s): 小亀浩市
  • Journal Title: 血液フロンティア Volume: 25 Pages: 51-57
  • NAID: 130004920625
• [Journal Article] ターゲット領域の異常を見逃さないゲノム定量PCR法の確立 (2014)
  • Author(s): 樋口（江浦）由佳，小亀浩市，宮田敏行
  • Journal Title: 日本血栓止血学会誌 Volume: 25 Pages: 615-618
• [Journal Article] TTPの分子診断 (2014)
  • Author(s): 小亀浩市
  • Journal Title: 日本血栓止血学会誌 Volume: 25 Pages: 689-696
  • NAID: 130004847353
• [Journal Article] Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy. (2014)
  • Author(s): Tanaka H, Tenkumo C, Mori N, Kokame K, Fujimura Y, Hata T.
  • Journal Title: J Obset Gynaecol Res. Volume: 40 Issue: 1 Pages: 247-249
  • DOI: 10.1111/jog.12125
  • Peer Reviewed
• [Journal Article] A first bout of thrombotic thrombocytopenic purpura triggered by herpes simplex infection in a 45-year-old nulliparous female with Upshaw-Schulman syndrome (2014)
  • Author(s): Masanobu Morioka, Masanori Matsumoto, Makoto Saito, Koichi Kokame, Toshiyuki Miyata, and Yoshihiro Fujimura
  • Journal Title: Blood Transfus. Volume: 12
  • Peer Reviewed
• [Journal Article] NDRG1 deficiency attenuates fetal growth and the intrauterine response to hypoxic injury (2014)
  • Author(s): Jacob Larkin, Baosheng Chen, Xiao-Hua Shi, Takuya Mishima, Koichi Kokame, Yaacov Barak, and Yoel Sadovsky
  • Journal Title: Endocrinology Volume: 155 Issue: 3 Pages: 1099-1106
  • DOI: 10.1210/en.2013-1425
  • Peer Reviewed
• [Journal Article] Candidate gene analysis using genomic quantitative PCR: identification of ADAMTS13 large deletions in two patients with Upshaw-Schulman syndrome. (2014)
  • Author(s): Eura Y, Kokame K, Takafuta T, Tanaka R, Kobayashi H, Ishida F, Hisanaga S, Matsumoto M, Fujimura Y, Miyata T.
  • Journal Title: olecular Genetics & Genomic Medicine. Volume: 2 Issue: 3 Pages: 240-244
  • DOI: 10.1002/mgg3.64
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Nonsynonymous mutations in three anticoagulant genes in Japanese patients with adverse pregnancy outcomes. (2014)
  • Author(s): Neki R, Miyata T, Fujita T, Kokame K, Fujita D, Isaka S, Ikeda T, Yoshimatsu J
  • Journal Title: Thromb Res Volume: 133 Issue: 5 Pages: 914-918
  • DOI: 10.1016/j.thromres.2014.02.012
  • Peer Reviewed / Open Access
• [Journal Article] 血栓性血小板減少性紫斑病 (2014)
  • Author(s): 藤村吉博，松本雅則，石西綾美，八木秀男，小亀浩市，宮田敏行
  • Journal Title: 臨床血液 Volume: 55 Pages: 93-104
  • NAID: 130004920625
• [Journal Article] Upshaw-Schulman症候群のADAMTS13遺伝子解析 (2014)
  • Author(s): 小亀浩市，樋口（江浦）由佳
  • Journal Title: 細胞 Volume: 46 Pages: 61-63
• [Journal Article] Crystal structure and enzymatic activity of an ADAMTS13 mutant with the East Asian-specific P475S polymorphism. (2013)
  • Author(s): Masashi Akiyama, Daisuke Nakayama, Soichi Takeda, Koichi Kokame, Junichi Takagi, and Toshiyuki Miyata.
  • Journal Title: J. Thromb. Haemost. Volume: (In press) Issue: 7 Pages: 1399-1406
  • DOI: 10.1111/jth.12279
  • Peer Reviewed
• [Journal Article] Up-regulation of endothelial nitric oxide synthase (eNOS), silent mating type information regulation 2 homologue 1 (SIRT1) and autophagy-related genes by repeated treatments with resveratrol in human umbilical vein endothelial cells (2013)
  • Author(s): Yoshie Takizawa, Yukiko Kosuge, Hiroyo Awaji, Emi Tamura, Ayako Takai, Takaaki Yanai, Reiko Yamamoto, Koichi Kokame, Toshiyuki Miyata, Rieko Nakata, and Hiroyasu Inoue
  • Journal Title: Brit. J. Nutr. Volume: 110 Issue: 12 Pages: 2150-2155
  • DOI: 10.1017/s0007114513001670
  • Peer Reviewed
• [Journal Article] The Satb1 protein directs hematopoietic stem cell differentiation toward lymphoid lineages. (2013)
  • Author(s): Satoh Y, Yokota T, Sudo T, Kondo M, Lai A, Kincade PW, Kouro T, Iida R, Kokame K, Miyata T, Habuchi Y, Matsui K, Tanaka H, Matsumura I, Oritani K, Kohwi-Shigematsu T, Kanakura Y.
  • Journal Title: Immunity Volume: 38 Issue: 6 Pages: 1105-1115
  • DOI: 10.1016/j.immuni.2013.05.014
  • Peer Reviewed
• [Journal Article] Long term follow up of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndorome) on hemodialysis for 19 years: a case report (2013)
  • Author(s): Mise K, Ubara Y, Matsumoto M, Sumida K, Hiramatsu R, Hasegawa E, Yamanouchi M, Hayami N, Suwabe T, Hoshino J, Sawa N, Ohashi K, Kokame K, Miyata T, Fujimura Y, Takaichi K.
  • Journal Title: BMC Nephrol. Volume: 14 Issue: 1 Pages: 156-156
  • DOI: 10.1186/1471-2369-14-156
  • Peer Reviewed
• [Journal Article] A deficiency of Herp, an endoplasmic reticulum stress protein, suppresses atherosclerosis in apoE knockout mice by attenuating inflammatory responses (2013)
  • Author(s): Shohei Shinozaki, Tsuyoshi Chiba, Koichi Kokame, Toshiyuki Miyata, Eiji Kaneko, and Kentaro Shimokado
  • Journal Title: PLoS One Volume: 8 Issue: 10 Pages: e75249-e75249
  • DOI: 10.1371/journal.pone.0075249
  • Peer Reviewed
• [Journal Article] Auto-adaptive ER-associated degradation defines a pre-emptive unfolded protein response pathway (2013)
  • Author(s): Riccardo Bernasconi, Carmela Galli, Koichi Kokame, and Maurizio Molinari
  • Journal Title: Mol. Cell Volume: 52 Issue: 6 Pages: 783-793
  • DOI: 10.1016/j.molcel.2013.10.016
  • Peer Reviewed
• [Journal Article] ADAMTS13 activity and genetic mutations in Japanese subjects. (2013)
  • Author(s): Toshiyuki Miyata, Koichi Kokame, Masanori Matsumoto, and Yoshihiro Fujimura.
  • Journal Title: Haemostaselolgie Volume: 33 Issue: 02 Pages: 131-137
  • DOI: 10.5482/hamo-12-11-0017
  • Peer Reviewed
• [Journal Article] ADAMTS13と血栓性血小板減少性紫斑病 (2013)
  • Author(s): 小亀浩市
  • Journal Title: 循環器病研究の進歩 Volume: 34 Pages: 69-75
• [Presentation] 先天性TTP／USSの遺伝子解析の現状 (2016)
  • Author(s): 小亀浩市
  • Organizer: 第10回日本血栓止血学会SSCシンポジウム
  • Place of Presentation: 東京
  • Year and Date: 2016-02-20
  • Invited
• [Presentation] 植え込み型補助人工心臓が血中von Willebrand factorに与える影響に関する研究 (2015)
  • Author(s): 水野敏秀, 内藤敬嗣, 樋口（江浦）由佳, 小亀浩市, 宮田敏行, 築谷朋典, 武輪能明, 巽英介
  • Organizer: 第53回人工臓器学会大会
  • Place of Presentation: 東京
  • Year and Date: 2015-11-19
• [Presentation] 定常流型左室補助人工心臓に用いる自己心拍同期回転数制御システムがvon Willebrand factorに及ぼす影響 (2015)
  • Author(s): 内藤敬嗣, 西村隆, 岸本諭, 水野敏秀, 武輪能明, 樋口（江浦）由佳, 小亀浩市, 宮田敏行, 小野稔, 巽英介
  • Organizer: 第53回人工臓器学会大会
  • Place of Presentation: 東京
  • Year and Date: 2015-11-19
• [Presentation] 野生型プロテインＳおよびK196E変異体の発現精製と機能解析 (2015)
  • Author(s): 丸山慶子, 小亀浩市, 秋山正志, 宮田敏行
  • Organizer: 第77回日本血液学会学術集会
  • Place of Presentation: 金沢
  • Year and Date: 2015-10-16
• [Presentation] Influence of a rotational speed modulation of an implantable continuous-flowleft ventricular assist device (EVAHEART) on von Willebrand factor dynamics (2015)
  • Author(s): Noritsugu Naito, Toshihide Mizuno, Takashi Nishimura, Satoru Kishimoto, Yoshiaki Takewa, Yuka Eura, Koichi Kokame, Toshiyuki Miyata, Minoru Ono, and Eisuke Tatsumi
  • Organizer: The 23rd Annual Meeting of the International Society for Rotary Blood Pumps (ISRBP)
  • Place of Presentation: Dubrovnik
  • Year and Date: 2015-09-27
  • Int'l Joint Research
• [Presentation] 日本人の非典型溶血性尿毒症症候群患者の遺伝子解析 補体系因子とDGKEの遺伝子変異 (2015)
  • Author(s): 宮田敏行, 加藤秀樹, 内田裕美子, 吉田瑶子, 小亀浩市, 福岡利仁, 要伸也, 大田敏之, 浦山耕太郎, 藤永周一郎, 櫻谷浩志, 喜瀬智郎, 渡邉栄三, 織田成人, 永田裕子, 玉井宏史, 小松真太郎, 前沢浩司, 川村尚久, 永野幸治, 河野智康, 松本雅則, 藤村吉博, 南学正臣
  • Organizer: 第52回日本補体学会学術集会
  • Place of Presentation: 名古屋
  • Year and Date: 2015-08-21
• [Presentation] ADAMTS13 assays, the need for multicenter studies (2015)
  • Author(s): Koichi Kokame, and Johanna A. Kremer Hovinga
  • Organizer: The 61th Annual Scientific and Standardization Committee Meeting
  • Place of Presentation: Toronto
  • Year and Date: 2015-06-20
  • Int'l Joint Research / Invited
• [Presentation] Development of ELISA system for detection of protein S K196E mutation, a genetic risk factor for venous thromboembolism (2015)
  • Author(s): Keiko Maruyama, Masashi Akiyama, Koichi Kokame, Akiko Sekiya, Eriko Morishita, and Toshiyuki Miyata
  • Organizer: XXV Congress of the International Society on Thrombosis and Haemostasis
  • Place of Presentation: Toronto
  • Year and Date: 2015-06-20
  • Int'l Joint Research
• [Presentation] Characteristics and outcomes of patients with Upshaw-Schulman syndrome receiving maintenance hemodialysis due to chronic renal failure (2015)
  • Author(s): Masanori Matsumoto, Ayami Isonishi, Koichi Kokame, Masaki Hayakawa, Hideo Yagi, Toshiyuki Miyata, and Yoshihiro Fujimura
  • Organizer: XXV Congress of the International Society on Thrombosis and Haemostasis
  • Place of Presentation: Toronto
  • Year and Date: 2015-06-20
  • Int'l Joint Research
• [Presentation] Hereditary TTP - a young patient population with high prevalence of arterial thromboembolic events: first results from the hereditary TTP registry (2015)
  • Author(s): Magnus Mansouri, Masanori Matsumoto, Zuzana Cermakova, Kenneth D. Friedman, James N. George, Ingrid Hrachovinova, Paul N. Knobl, Koichi Kokame, Anne Sophie von Krogh, Reinhard Schneppenheim, Sara K. Vesley, Yoshihiro Fujimura, Bernhard Lammle, and Johanna A. Kremer Hovinga
  • Organizer: XXV Congress of the International Society on Thrombosis and Haemostasis
  • Place of Presentation: Toronto
  • Year and Date: 2015-06-20
  • Int'l Joint Research
• [Presentation] 補助人工心臓装着による高分子量VWFマルチマーの減少は循環開始直後に始まる (2015)
  • Author(s): 樋口（江浦）由佳, 小亀浩市, 水野敏秀, 巽英介, 宮田敏行
  • Organizer: 第37回日本血栓止血学会学術集会
  • Place of Presentation: 甲府
  • Year and Date: 2015-05-21
• [Presentation] 先天性TTP（USS）の分子診断 (2015)
  • Author(s): 小亀浩市
  • Organizer: 第9回日本血栓止血学会SSCシンポジウム
  • Place of Presentation: 東京
  • Year and Date: 2015-02-28
  • Invited
• [Presentation] αIIb(R990W), a constitutive activating mutation of integrin αIIbβ3, knock-in mice show macrothrombocytopenia with impairment of platelet function (2014)
  • Author(s): Kazunobu Kiyomizu, Hirokazu Kashiwagi, Shinji Kunishima, Fumiaki Banno, Hisashi Kato, Youichiro Morikawa, Seiji Tadokoro, Koichi Kokame, Shigenori Honda, Toshiyuki Miyata, Yuzuru Kanakura, Yoshiaki Tomiyama
  • Organizer: The 56th American Society of Hematology (ASH) Annual Meeting
  • Place of Presentation: San Francisco
  • Year and Date: 2014-12-06 – 2014-12-09
• [Presentation] プロテインS K196E（PS徳島）変異検出ELISA法の開発 (2014)
  • Author(s): 丸山慶子，秋山正志，小亀浩市，森下英理子，宮田敏行
  • Organizer: 第76回日本血液学会学術集会
  • Place of Presentation: 大阪
  • Year and Date: 2014-10-31 – 2014-11-02
• [Presentation] HUVECにおけるレスベラトロールによるeNOS、SIRT1、オートファジー関連遺伝子の発現誘導 (2014)
  • Author(s): 滝澤祥恵，小菅由希子，淡路比呂代，田村恵美，高井綾子，矢内隆章，山本玲子，小亀浩市，宮田敏行，井上裕康
  • Organizer: 第87回日本生化学会大会
  • Place of Presentation: 京都
  • Year and Date: 2014-10-15 – 2014-10-18
• [Presentation] TTP患者の遺伝子解析 (2014)
  • Author(s): 小亀浩市
  • Organizer: 第36回日本血栓止血学会学術集会
  • Place of Presentation: 大阪
  • Year and Date: 2014-05-29 – 2014-05-31
  • Invited
• [Presentation] デジタルPCRを用いたADAMTS13遺伝子変異のヘテロ接合性の解析 (2014)
  • Author(s): 小亀浩市，樋口（江浦）由佳，松本雅則，藤村吉博，宮田敏行
  • Organizer: 第36回日本血栓止血学会学術集会
  • Place of Presentation: 大阪
  • Year and Date: 2014-05-29 – 2014-05-31
• [Presentation] ダイレクトシークエンシング，定量PCR，次世代シークエンシングを用いたTTP患者の遺伝子解析 (2014)
  • Author(s): 樋口（江浦）由佳，小亀浩市，高蓋寿朗，田中亮二郎，小林光，石田文宏，久永修一，松本雅則，藤村吉博，宮田敏行
  • Organizer: 第36回日本血栓止血学会学術集会
  • Place of Presentation: 大阪
  • Year and Date: 2014-05-29 – 2014-05-31
• [Presentation] 深部静脈血栓症モデルを用いた日本人の血栓症モデルマウスの解析 (2014)
  • Author(s): 坂野史明，田嶌優子，小亀浩市，宮田敏行
  • Organizer: 第36回日本血栓止血学会学術集会
  • Place of Presentation: 大阪
  • Year and Date: 2014-05-29 – 2014-05-31
• [Presentation] インテグリンαIIbβ3活性化変異, αIIb(R990W)ノックインマウスの解析 (2014)
  • Author(s): 清水一亘，柏木浩和，森川陽一郎，加藤恒，田所誠司，坂野史明，小亀浩市，本田繁則，金倉譲，宮田敏行，冨山佳昭
  • Organizer: 第36回日本血栓止血学会学術集会
  • Place of Presentation: 大阪
  • Year and Date: 2014-05-29 – 2014-05-31
• [Presentation] VWF切断酵素ADAMTS13の基礎知識 (2014)
  • Author(s): 小亀浩市
  • Organizer: 第62回日本輸血・細胞治療学会総会
  • Place of Presentation: 奈良
  • Year and Date: 2014-05-15 – 2014-05-17
  • Invited
• [Presentation] Analysis of mice carrying northeast Asian-specific genetic mutations in thrombosis (2014)
  • Author(s): Fumiaki Banno, Yuko Tashima, Toshiyuki Kita, Yasuyuki Matsuda, Hiroji Yanamoto, Kochi Kokame, Toshiyuki Miyata
  • Organizer: The 18th International Vascular Biology Meeting
  • Place of Presentation: Kyoto
  • Year and Date: 2014-04-14 – 2014-04-17
• [Presentation] ADAMTS13の正常な分泌にはシクロフィリンBによるプロリン残基異性化が必要である (2013)
  • Author(s): 小亀浩市，秋山正志，宮田敏行
  • Organizer: 第35回日本血栓止血学会学術集会
  • Place of Presentation: 山形
• [Presentation] プロテインS徳島（PS-K196E）変異ノックインマウスの樹立と解析 (2013)
  • Author(s): 坂野史明，喜多俊行，柳本広二，田嶌優子，小亀浩市，宮田敏行
  • Organizer: 第35回日本血栓止血学会学術集会
  • Place of Presentation: 山形
• [Presentation] 妊娠を契機に診断された新規の遺伝子変異を伴うUpshaw-Schulman症候群（USS）の一例 (2013)
  • Author(s): 小堺貴司，森山雅人，布施一郎，柴崎康彦，増子正義，瀧澤淳，鳥羽健，吉田邦彦，小亀浩市，宮田敏行，松本雅則，藤村吉博，曽根博仁
  • Organizer: 第35回日本血栓止血学会学術集会
  • Place of Presentation: 山形
• [Presentation] マウス急性心筋梗塞モデルにおけるADAMTS13の心筋保護作用 (2013)
  • Author(s): 土井政明，松井英人，竹田征治，斎藤能彦，武田麻衣子，松成泰典，西尾健治，嶋緑倫，坂野史明，秋山正志，小亀浩市，宮田敏行，杉本充彦
  • Organizer: 第35回日本血栓止血学会学術集会
  • Place of Presentation: 山形
• [Presentation] Quantitative PCR-based analysis of ADAMTS13 genetic defects (2013)
  • Author(s): Koichi Kokame
  • Organizer: The 59th Annual Scientific and Standardization Committee Meeting
  • Place of Presentation: Amsterdam, The Netherlands
  • Invited
• [Presentation] Quantitative PCR assay demonstrated exon deletions of ADAMTS13 in two unrelated patients with Upshaw-Schulman syndrome (2013)
  • Author(s): Yuka Eura, Koichi Kokame, Toshiro Takafuta, Ryojiro Tanaka, Hikaru Kobayashi, Fumihiro Ishida, Shuichi Hisanaga, Masanori Matsumoto, Yoshihiro Fujimura, and Toshiyuki Miyata
  • Organizer: XXIV Congress of the International Society on Thrombosis and Haemostasis
  • Place of Presentation: Amsterdam, The Netherlands
• [Presentation] Exacerbated venous thromboembolism in mice with the protein S Tokushima mutation (2013)
  • Author(s): Fumiaki Banno, Toshiyuki Kita, Hiroji Yanamoto, Yuko Tashima, Koichi Kokame, and Toshiyuki Miyata
  • Organizer: XXIV Congress of the International Society on Thrombosis and Haemostasis
  • Place of Presentation: Amsterdam, The Netherlands
• [Presentation] 血栓性血小板減少性紫斑病患者のADAMTS13遺伝子に伏在していた変異の発見：ゲノムDNAを用いた定量PCR法の開発 (2013)
  • Author(s): 樋口（江浦）由佳，小亀浩市，松本雅則，藤村吉博，宮田敏行
  • Organizer: 第18回日本病態プロテアーゼ学会学術集会
  • Place of Presentation: 豊中
• [Presentation] 日本における先天性ADAMTS13欠損症の遺伝子解析 (2013)
  • Author(s): 樋口（江浦）由佳，小亀浩市，松本雅則，藤村吉博，宮田敏行
  • Organizer: 第86回日本生化学会大会
  • Place of Presentation: 横浜
• [Presentation] A long-term phenotype analysis of 51 patients with Upshaw–Schulman syndrome in Japan, with special references to pregnancy and renal failure that requires hemodialysis (2013)
  • Author(s): Yoshihiro Fujimura, Yumi Yoshii, Masanori Matsumoto, Ayami Isonishi, Masaki Hayakawa, Yoko Yoshida, Hideo Yagi, Koichi Kokame, and Toshiyuki Miyata
  • Organizer: The 55th ASH Annual Meeting and Exposition
  • Place of Presentation: New Orleans, USA
• [Remarks] 国立循環器病研究センター 研究所 分子病態部
• [Remarks] 国立循環器病研究センター 研究所 分子病態部
  • URL: http://www.ncvc.go.jp/res/divisions/etiology/index.html