Prof. Fujimura’s profile

Dr. Yoshihiro Fujimura, born in 25 March 1949 at Mie prefecture, graduated from Nara Medical University in 1973. After an initial basic clinical training as a pediatrician at Nara Medical University Hospital, he joined to a staff doctor at Osaka National Hospital under direction of the late Dr. Keiichiro Yoshioka and Prof. Akira Yoshioka, where Dr. Fujimura had special interests on the clinical research in congenital and acquired bleeding disorders. In 1978, he moved to Department of Pediatrics, Nara Medical University, and continued to work as a pediatric hematologist for the following 6 years, under the direction of the late Prof. Hiromu Fukui.

 During 1984-1986, he served as a NIH-Fogarty International Research Fellow at the Scripps Clinic and Research Foundation (La Jolla, CA), where he studied on the structure-function relationship of von Willebrand factor (VWF), a macromolecular plasma glycoprotein required for primary hemostasis, and more specifically to the identification of platelet glycoprotein (GP) Ib-binding domain of VWF, under direction of the late Prof. Theodore S. Zimmerman and Prof. Zaverio M. Ruggeri.

 In 1987, Dr. Fujimura returned to his alma mater as a staff doctor of Blood Transfusion Medicine, and in 1998 he was promoted to a professor in the same institution. Because he had a distinguished background for VWF-research, during the following 10 year his work has been engaged in the basic and clinical research on VWF-cleaving protease, now known as ADAMTS13. Through these studies, he and his co-workers established the chromogenic ADAMTS13-act-ELISA, a rapid and sensitive assay for ADAMTS13 activity, which is now used as with a standard commercial kit globally and enabled in his laboratory a huge registry of 919 patients with thrombotic microangiopathies across Japan. Because of these achievements, he received Ba¨lz prize (silver medal) in 2008 with his five colleagues Drs. Matsumoto M, Uemura M, Sugimoto M, Kokame K, and Miyata T.


1. Kosugi N, Tsurutani Y, Isonishi A, Hori Y, Matsumoto M, Fujimura Y. Influenza A infection triggers thrombotic thrombocytopenic purpura by producing the anti-ADAMTS13 IgG inhibitor. Intern Medicine (in press), 2010.
2. Okano E, Ko S, Matsumoto M, Fujimura Y, Nakajima Y. ADAMTS13 activity decreases after hepatectomy, reflecting a postoperative liver function. Hepato-gastroenterology (in press), 2010.
3. Sato A, Hoshi Y, Onuma M, Sato R, Tsunematsu Y, Isonishi A, Matsumoto M, Fujimura Y, Imaizumi M. A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13. Pediatric Hematology-Oncology (in press), 2010.
4. Uemura M, Fujimura Y, Ko S, Matsumoto M, Nakajima Y, Fukui H. Pivotal role of ADAMTS13 function in liver diseases. Int J Haematol (Review), 2010 Jan 7. [Epub ahead of print].
5. Fujimura Y, Matsumoto M. Registry of 919 patients with thrombotic microangiopathies across Japan: Database of Nara Medical University during 1998-2008. Intern Medicine 49: 7-15, 2010.

6. Ito N, Wada H, Matsumoto M, Fujimura Y, Murata M, Izuno T, Sugita M, Ikeda Y. National questionnaire survey of TMA. Int J Hematol 90: 328-335, 2009.
7. Watanabe N, Ikeda H, Kume Y, Satoh Y, Takai D, Tejima K, Mashima H, Tomiya T, Nagamine M, Noiri E, Omata M, Fujiwara K, Matsumoto M, Fujimura Y, Yatomi Y. Increased production of ADAMTS13 in hepatic stellate cells leads to enhanced plasma ADAMTS13 activity in rat models of cholestasis and steatohepatitis. Thromb Haemost 102: 371-378, 2009.
8. Matsuyama T, Kuwana M, Matsumoto M, Isonishi A, Inokuma S, Fujimura Y. Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases. Thromb Haemost 102: 371-378, 2009.
9. Kobayashi T, Wada H, Usui M , Sakurai H , Matsumoto T, Nobori T, Katayama N, Uemoto S, Ishizashi H, Matsumoto M, Fujimura Y, Isaji S. Decreased ADAMTS13 levels in patients with living donor liver transplantation. Thromb Res 124: 541-545, 2009.
10. Takemoto Y, Kawata H, Soeda T, Imagawa K, Somekawa S, Takeda Y, Uemura S, Matsumoto M, Fujimura Y, Jo J, Kimura Y, Tabata Y, Saito Y. Human placental ectonucleoside triphosphate diphosphohydrolase gene transfer via gelatin-coated stents prevents in-stent thrombosis. Arterioscl Thromb Vas Biology 29: 857-862, 2009.
11. Shimizu M, Nomura S, Ishii K, Mohri Y, Umei N, Suzuki S, Matsuo Y, Isonishi A, Matsumoto M, Urase F, Maeda Y, Fujimura Y. The significence of ADAMTS13 in a patient with thrombotic thrombocytopenic purpura complicated by autoimmune hepatitis. Thromb Haemost (Letters to the Editor) 101: 599-600, 2009.
12.Fujimura Y, Matsumoto M, Kokame K, Isonishi A, Soejima K, Akiyama N, Tomiyama J, , Natori K, Kuranishi Y, Imamura Y, Inoue N, Higasa S, Seike M, Kozuka T, Hara M, H. Wada, Murata M, Ikeda Y, Miyata T, George JN. Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients. Br J Haematol 144: 742-754, 2009.
13. Fujimura Y, Matsumoto M. Thrombotic thrombocytopenic purpura: Results of the patients with thrombotic microangiopathies across Japan by ADAMTS13 analysis during 1998-2008. Vox Sanguinis (Review) 4: 174-181, 2009.
14. Zakarija A, Kwaan H-C, Moake JL, Bandarenko N, Pandey DK, McKoy JM, Yarnold PR, Raisch DW, Winters JL, Raife TJ, Cursio JF, Ha Luu T-H, Richey EA, Fisher M, Ortel TL, Tallman MS, Zheng XL, Matsumoto M, Fujimura Y Bennett CL. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic prupura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigillance findings (1989-2008). Kidney International 112: 520-524, 2009.

15. Kanno M, Nakamura S, Kawahara M, Ueno S, Nakamine H, Matsumoto M, Fujimura Y. Chemotherapy-resistent intravascular lymphoma accompanied by ADAMTS13 inhibitor successfully treated with rituximab. Int J Hematol 88: 345-347, 2008.
16. Tripodi A, Peyvandi F, Chantarangkul V, Palla R, Afrasiabi A, Canciani MT, Chung DW, Ferrari S, Fujimura Y, Karimi M, Kokame K, Kremer-Hovinga JA, La¨mmle B, De Meyer SF, Plaimauer B, Vanhoorelbeke K, Varadi K, Mannucci PM. Second international collaborative study evaluating performance characteristics of methods measuring the von Willebrand factor cleaving protease (ADAMTS-13). J Thromb Haemost 6: 1534-1541, 2008.
17.Morioka C, Uemura M, Matsuyama T, Matsumoto M, Kato S, Ishikawa M, Ishizashi H, Sawai M, Yoshida M, Mitoro A, Yamao J, Tsujimoto T, Yoshiji H, Urizono Y, Hata M, Nishio K, Okuchi K, Fujimura Y, Fukui H. Plasma ADAMTS13 activity parallels the APACHE II score, reflecting an early prognostic indicator for patients with severe acute pancreatitis. Scand J Gatroenteronology 43: 1387-1396, 2008.
18. Horii M, Uemura S, Uemura M, Matsumoto M, Ishizashi H, Imagawa K, Iwama H, Takeda Y, Kawata H, Nakajima T, Fujimura Y, Saito Y. Acute myocardial infarction as a systemic prothrombotic condition evidenced by increased von Willebrand factor protein over ADAMTS13 activity in coronary and systemic circulation. Heart and Vessels 23: 301-307, 2008.
19.Furukoji E, Tanaka N, Yamashita A, Matsumoto M, Fujimura Y, Yamamoto R, Tamura S, Asada Y. Ecto-nucleoside triphosphate diphosphohydrolase inhibits ATP- and ADP-induced vasoconstriction. Thromb Res 121: 583-585, 2008.
20. Uemura M, Fujimura Y, Matsumoto M, Ishizashi H, Kato S, Matsuyama T, Isonishi A, Ishikawa M, Yagita M, Yoshiji H, Tsujimoto T, Fukui H. Comprehensive analysis of ADAMTS13 in patients with liver cirrhosis. Thromb Haemost 99:1019-1029, 2008.
21. Kato K, Kobayashi C, Katayama Y, Moriyama N, Shiono J, Kudo K, Koide K, Aoki K, Fujisawa K, Okada M, Matsumoto M, Fujimura Y, Tsuchida M. A one-month-old boy with acute idiopathic thrombocytopenic purpura complicated with intracranial hemorrhage in association with minor head trauma. J Ped Hematol Oncology (in press), 2008.
22.Kokame M, Aoyama Y, Matsumoto M, Fujimura Y, Miyata T. Inherited and de novo mutations of ADAMTS13 in a patient with Upshaw-Schulman syndrome. J Thromb Haemost (Letters to the Editor) 6: 213-215, 2008.
23.Shida S, Nishio K, Sugimoto M, Mizuno T, Hamada M, Kato S, Matsumoto M, Okuchi K, Fujimura Y, Yoshioka A. Functional imaging of shear-dependent activity of ADAMTS13 in regulating mural thrombis growth under whole blood flow conditions. Blood 111: 1295-1298 2008.
24.Uemura M, Fujimura Y, Matsuyama T, Matsumoto M, Ishikawa M, Ishizashi H, Kato S, Tsujimoto T, Yoshiji H, Morioka C, Fukui H. Potential role of ADAMTS13 in the progression of alcoholic hepatitis. Open Gastroentenology (Review) 1: 188-196, 2008.

25.Matsumoto M, Kawa K, Uemura M, Kato S, Ishizashi H, Isonishi A, Yagi H, Park Y-D, Takeshima Y, Kosaka Y, Hara H, Kai S, Kanamaru A, Fukuhara S, Hino M, Sako M, Hiraoka A, Ogawa H, Hara J, Fujimura Y. Prophylactic fresh frozen plasma infusion may prevent the development of hepatic VOD after stem cell transplantation via ADAMTS13-mediated restoration of von Willebrand factor plasma levels. Bone Marrow Transplant 40: 251-259, 2007.
26.Bennett CL, Banjamin K, Zakarija A, Bandarenko N, Pandey DK, Buffie CG, McKoy JM, Tevar AD, Cursio JF, Yarnold PR, Kwaan HC, De Masi D, Sarode R, Raife TJ, Kiss JE, Raisch DW, Davidson C, Sadler JE, Ortel TL, Zheng XL, Kato S, Matsumoto M, Uemura M, Fujimura Y. Two mechanistic pathways for thienopyridine-associated thrombotic thrombocytopenic purpura: A report from the Surveillance, Epidemiology, and Risk Factors for Thrombotic Thrombocytopenic Purpura (SERF-TTP) research group and the Research on Adverse Drug Events and Reports (RADAR) project. J Am Coll Cardiol 50:1138-1143, 2007.
27.Yagi H, Ito S, Kato S, Hiura H, Matsumoto M, Fujimura M. Plasma levels of ADAMTS13 antigen, determined by an enzyme immunoassay using the neutralizing monoclonal antibody, parallel to those of the activity. Int J Hematol 85: 403-407, 2007.
28.Ito S, Okuyama K, Nakamura T, Tetanishi J, Saito K, Matsumoto M, Fujimura Y, Aihara Y, Yokota S. Intravenous gamma-globulin for thrombotic microangiopathy of unknown etiology. Pediatric Nephrology 22: 301-305, 2007.
29.Ishizashi H, Yagi H, Matsumoto M, Soejima K, Nakagaki T, Fujimura Y. Quantitative western blot analysis of plasma ADAMTS13 antigen in patients with Upshaw-Schulman syndrome. Thromb Res 120: 381-386, 2007.
30.Morishita T, Matsumoto M, Honoki K, Yoshida A, Takakura Y, Fujimura Y. Successful treatment of primitive neuroectodermal tumor-associated microangiopathy with multiple bone metastasis. Jap J Clin Oncology 37: 66-69, 2007.
31.Kobayashi T, Wada H, Kamikura Y, Matsumoto T, Mori Y, Kaneko T, Nobori T, Matsumoto M, Fujimura Y, Shiku H. Decreased ADAMTS13 activity in plasma from patients with thrombotic thrombocytopenic purpura. Thromb Res 119: 447-452, 2007.
32.Fujimura Y, Matsumoto M, Yagi H. Thrombotic microangiopathy. Recent Advances in Thrombosis and Hemostasis 2008 (ed by Tanaka K, Davie EW). Springer-Verlag, pp 625-639, 2007.

33.Matsuyama T, Uemura M, Ishikawa M, Matsumoto M, Ishizashi H, Kato S, Morioka C, Fujimoto M, Kojima H, Yoshiji H, Takimura C, Fujimura Y, Fukui H. Increased von Willebrand factor over decreased ADAMTS13 activity may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis. Alcohol Clin Exp Res 31: 27S-35S, 2006.
34.Kitano K, Gibo Y, Kamijo A, Furuta K, Oguchi S, Joshita S, Takahashi Y, Ishida F, Matsumoto M, Uemura M, Fujimura Y. Thrombotic thrombocytopenic purpura associated with pegylated-interferon alpha-2a by an ADAMTS13 inhibitor in a patient with chronic hepatitis C. Hematologica (on line) 91(1) 5-7, 2006.
35.Kato S, Matsumoto M, Matsuyama T, Isonishi A, Hiura H, Fujimura Y. Novel monoclonal antibody-based immunoassay for determining plasma levels of ADAMTS13 activity. Transfusion 46: 1444-1452, 2006.
36.Sato T, Hanaoka R, Ohshima M, Miwa Y, Okazaki Y, Uajima N, Ishizashi H, Matsumoto M, Fujimura M, Inokuma S. Analysis of ADAMTS13 activity and its inhibitor in patients with thrombotic thrombocytopenic purpura secondary to connective tissue diseases: Observations in a single hospital. Clin Exp Rheumatol (Letters to the Editor) 24:454-455, 2006.
37.Ko S, Okano H, Kanehiro H, Matsumoto M, Ishizashi H, Uemura M, Fujimura Y, Tanaka K, Nakajima Y. Plasma ADAMTS13 activity may predict early adverse events in living donor liver transplantation: Observations in 3 cases. Liver Transplant 12:859-869, 2006.
38.Shibagaki Y, Matsumoto M, Kokame K, Ohba S, Miyata T, Fujimura Y, Fujita T. Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure. Nephrol Dial Transplant 21: 1289-1292, 2006.

39.Uemura M, Matsuyama T, Fujimoto M, Kojima H, Sakurai S, Ishii S, Toyohara M, Yamazaki M, Yoshiji H, Yamao J, Matsumoto M, Ishizashi H, Fujimura Y, Fukui H. Decreased activity of plasma ADAMTS13 may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis. Alcohol Clin Exp Res 29: 264-71, 2005.
40.Matsumoto M, Kawaguchi S, Ishizashi H, Yagi H, Iida J, Sakaki T, Fujimura Y. Platelets treated with ticlopidine are less reactive to unusually large VWF multimers than are those treated with aspirin under high shear stress. Pathophy Haemost Thromb 34: 35-40, 2005.
41.Uemura M, Tatsumi K, Matsumoto M, Fujimoto M, Matsuyama T, Ishikawa M, Iwamoto T, Mori T, Wanaka A, Fukui H, Fujimura Y. Localization of ADAMTS13 to the stellate cells of human liver. Blood 106: 922-924, 2005.
42.Kosugi S, Matsumoto M, Ohtani Y, Take H, Ishizashi H, Fujimura Y, Kuyama J. Rituximab provided long-term remission in a patient with refractory relapsing thrombotic thrombocytopenic purpura. Int J Hematol 81: 433-436, 2005.
43. Fujisaki K, Matsutani K, Yoshimitsu T, Nakanishi K, Matsumoto M, Yagi H, Ishizashi H, Fujimura Y, Takeda K, Hirakata H, Iida M. Thrombotic thrombocytopenic purpura associated with polyarthritis nodosa: demonstration of the inhibitor against von Willebrand factor-cleaving protease. Clinical Nephrology 64: 305-310, 2005.
44.Sugimoto T, Saigo K, Shin T, Kaneda Y, Manabe N, Narita H, Wakuya J, Imoto S, Murayama T, Matsumoto M, Fujimura Y, Nishimura R, Koizumi T, Kumagai S. Von Willebrand factor-cleaving protease activity remains at the intermediate level in thrombotic thrombocytopenic puroura. A chronic myelogenous case treated with interferon-α. Acta Haematologica 113:198-203, 2005.
45.Hatakeyama K, Hao H, Imamura T, Ishikawa T, Shibata Y, Fujimura Y, Eto T, Ogawa H, Asada Y. Decreased CD39 expression in coronary artherosclerotic lesions is implicated in plaque instability and thrombus formation. Am J Cardiol 95: 632-635, 2005.
46.Furukoji E. Matsumoto M, Yamashita A, Yagi H, Sakurai Y, Marutsuka K, Hatakeyama K, Morishita K, Fujimura Y, Tamura S, Asada Y. Adenovirus-mediated transfer of human placental ecto-ATP diphosphohydrolase I to vascular smooth muscle cells suppresses platelet aggregation in vitro and arterial thrombus formation in vivo. Circulation 111: 808-815, 2005.
47.Yagita M, Uemura M, Nakamura T, Kunitomi A, Matsumoto M, Fujimura Y. Development of ADAMTS13 inhibitor in a patient with hepatitis C virus-related liver cirrhosis causes thrombotic thrombocytopenic puroura. J Hepatology (Letters to the Editor) 42: 420-421, 2005.
48.Fujimura Y. Down-regulation of ADAMTS13 activity by serine proteases. Blood (Inside Blood) 105: 911-912, 2005.

49.Kawahara M, Kanno M, Matsumoto M, Nanno H, Danno D, Murata K, Nakamura S, Fujimura Y, Ueno S. Diffuse neurodeficits in intravascular lymphomatosis with ADAMTS13 inhibitor. Neurology 63: 1731-1733, 2004.
50.Uchida T, Wada H, Mizutani M, Iwashita M, Ishihara H, Shibano T, Suzuki M, Matsubara Y, Soejima K, Matsumoto M, Fujimura Y, Ikeda Y, Murata M. Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura. Blood 104: 2081-2083, 2004.
51.Matsumoto M, Kokame K, Soejima K, Miura M, Hayashi S, Fujii Y, Iwai A, Ito E, Tsuji Y, Takada-Shitaka M, Iwadate M, Umeyama H, Yagi H, Ishizashi H, Banno F, Nakagaki T, Miyata T, Fujimura Y. Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome. Blood 103: 1305-1310, 2004.
52.Matsumoto M, Yagi H, Ishizashi H, Wada H, Fujimura Y. The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Seminar in Hematol 41: 68-74, 2004.
53.Kokame K, Matsumoto M, Fujimura Y, Miyata T. VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13. Blood 103: 607-612, 2004.

54.Fujimura Y. Is Upshaw-Schulman syndrome congenital TTP or HUS? Yes on the both. J Thromb Haemost (Letters to the Editor) 1: 2457-2458, 2003.
55.Soejima K, Matsumoto M, Kokame K, Yagi H, Ishizashi H, Maeda H, Nozaki C, Miyata T, Fujimura Y, Nakagaki T. ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage. Blood 102: 3232-3237, 2003.
56.Nakazawa Y, Hashikura Y, Ogino S, Urata K, Ikegami T, Terada M, Yagi H, Ishizashi H, Matsumoto M, Fujimura Y, Kawasaki S. Significance of measuring von Willebrand factor-cleaving protease activity in thrombotic microangiopathy after living donor liver transplantation. Liver Transplant 9: 1328-1333, 2003.
57.Sakurai Y, Shima M, Matsumoto M, Takatsuka H, Nishiya K, Kasuda S, Fujimura Y, Yoshioka A. Anticoagulant activity of M-LAO, L-amino acid oxidase purified from Agkistrodon halys blomhoffi, through selective inhibition of factor IX. Biochem Biophys Acta 1649: 51-57, 2003.
58.Wang J, Kimura T, Asada R, Harada S, Yokota S, Kawamoto Y, Fujimura Y, Tsuji T, Ikehara S, Sonoda Y. SCID-repopulating cell activity of human cord blood-derived CD34-negative cells assured by intra-bone injection. Blood 101:2924-2931, 2003.
59.Fujimura Y. TTP: VWF and ADAMTS-13. Int J Hematol (Review). pp77-83, 2003.

60.Sugimoto M, Matsui H, Mizuno T, Tsuji S, Miyata S, Matsumoto M, Matsuda M, Fujimura Y, Yoshioka A. Mural thrombus generation in type 2A and 2B von Willebrabd disease under high shear conditions. Blood 101: 915-920, 2002.
61.Yasuzawa-Amano S, Inoue M, Yoshida Y, Yagi H, Ishizashi H, Matsumoto M, Fujimura Y, Yoshioka A. Intensive plasma IgG removal therapy for severe thrombotic thrombocytopenic purpura. Int J Haematol 76: 474-476, 2002.
62.Ashida A, Nakamura H, Yoden A, Tamai H, Ishizashi H, Yagi H, Matsumoto M, Fujimura Y. Successful treatment of a young infant who developed high-titer inhibitors against VWF-cleaving protease (ADAMTS-13): Important discrimination from Upshaw-Schulman syndrome. Am J Hematol 71: 318-322, 2002.
63.Kokame K, Matsumoto M, Soejima K, Yagi H, Ishizashi H, Funato M, Tamai H, Konno M, Kamide K, Kawano Y, Miyata T, Fujimura Y. Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity. Proc Natl Acad Sci USA 99: 11902-11907, 2002.
64.Matsuda J, Sanaka T, Gohchi K, Matsui K, Uchida S, Matsumoto M, Fujimura Y. Occurrence of thrombotic thrombocytopenic purpura in a systemic lupus erythrematosus patient with antiphospholipid antibodies in association with a decreased activity of von Willebrand factor-cleaving protease. Lupus 11: 463-464, 2002.
65.Matsui T, Hamako J, Matsushita T, Nakayama T, Fujimura Y, Titani K. Binding site on human von Willebrand factor of bitiscetin, a snake venom-derived platelet aggregation inducer. Biochemistry USA 41: 7939-7946, 2002.
66.Park Y-D, Yoshioka A, Kawa K, Ishizashi H, Yagi H, Yamamoto Y, Matsumoto M, Fujimura Y. Impaired activity of plasma von Willebrand factor-cleaving protease may predict the occurrence of hepatic veno-occlusive disease after stem cell transplantation. Bone Marrow Transplant 29: 789-794, 2002.
67.Mori Y, Wada H, Gabazza EC, Minami N, Nobori T, Shiku H, Yagi H, Ishizashi H, Matsumoto M, Fujimura Y. Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity. Transfusion 42: 572-580, 2002.
68.Hayashi T, Yagi H, Suzuki H, Nonaka Y, Nomura T, Sakurai Y, Shibata M, Matsumoto M, Yamamoto Y, Fujimura Y. Low-dosage intravenous immunoglobulin in the management of a patient with acquired von Willebrand syndrome associated with monoclonal gammopathy of undetermined significance. Pathophys Haemost Thromb 32: 33-39, 2002.
69 .Orimo S, Ozawa E, Yagi H, Ishizashi H, Matsumoto M, Fujimura Y. Simple plasma exchange reduced autoantibody to von Willebrand factor-cleaving protease in a Japanese man with ticlopidine-associated thrombotic thrombocytopenic purpura. J Intern Med 251: 280-281, 2002.
70.Fujimura Y, Matsumoto M, Yoshioka A, Matsui T, Titani K. von Willebrand factor-cleaving protease and Upshaw-Schulman syndrome. In: Progress in Hematology. Int J Haematol (Review) 75: 25-34, 2002.

71.Yagi H, Konno M, Kinoshita S, Matsumoto M, Ishizashi H, Matsui T, Titani K, Fujimura Y. Plasma of patients with Upshaw-Schulman syndrome, a congenital deficiency of von Willebrand factor-cleaving protease activity, enhances the aggregation of normal platelets under high shear stress. Br J Haematol 115: 991-997, 2001.
72.Sakurai Y, Takatsuka H, Yoshioka A, Matsui T, Suzuki M, Titani K, Fujimura Y. Inhibition of human platelet aggregation by L-amino acid oxidase purified from Naja kaouthia venom. Toxicon 39: 1827-1833, 2001.
73.Sugio Y, Okamura T, Shimoda K, Matsumoto M, Yagi H, Ishizashi H, Niho Y, Inaba S, Fujimura Y. Ticlopidine-associated thrombotic thrombocytopenic purpura with an IgG-type inhibitor to von Willebrand factor-cleaving protease activity. Int J Haematol 74: 347-351, 2001.
74.Kinoshita S, Yoshioka A, Park Y-D, Ishizashi H, Konno M, Funado M, Matsui T, Titani K, Yagi H, Matsumoto M, Fujimura Y. Upshaw-Schulman syndrome revisited: A concept of congenital thrombotic thrombocytopenic purpura. Int J Haematol 74: 101-108, 2001.
75.Ito M, Hamako J, Sakurai Y, Matsumoto M, Fujimura Y, Suzuki M, Hashimoto K, titani K. Complete amino acid sequence of kaouthiagin, a novel cobra venom metalloproteinase with two disintegrin-like sequences. Biochemistry USA 40:4503-4511, 2001.
76.Yagi H, Narita N, Matsumoto M, Sakurai Y, Ikari H, Yoshioka A, Kita E, Ikeda Y, Titani K, Fujimura Y. Enhanced low shear stress-induced platelet aggregation by Shiga-like toxin 1 purified from Escherichia coli O157. Am J Hematol 66: 105-115, 2001
77.Takatsuka H、Sakurai Y、Yoshioka A、Kokubo T、Usami Y、Suzuki M、Titani K、Yagi H、Matsumoto M、Fujimura Y. Molecular characterization of L-amino acid oxidase from Agkistrodon halys blomhoffi with special reference to platelet aggregation. Biochem Biophys Acta 1544: 267-277, 2001.

78.Satoh K, Asazuma N, Yatomi H, Fujimura Y, Miura S, Titani K, Ozaki Y. Activation of protein-tyrosine kinase pathways in human platelets stimulated with the A1 domain of von Willebrand factor. Platelets 11: 171-176, 2000.
79.Nomura S, Imamura A, Okuno S, Komiyama N, Fujimura Y, Ikeda Y, Fukuhara S. Platelet-derived microparticles in patients with arteriosclerosis obliterans: Enhancement of high shear-induced microparticle generation by cytokines. Thromb Res 98: 257-268, 2000.
80.Taniuchi Y, Kawasaki T, Fujimura Y. The high molecular mass, glycoprotein Ib-binding protein Flavocetin-A induces only small platelet aggregation in vivo. Thromb Res 97: 69-75, 2000.
81.Yamamoto T, Moriwaki Y, Fujimura Y, Takahashi S, Tsutsumi Z, Tsutsui T, Higashino K, Hada T. Effect of TEI-6720, a xanthine oxidase inhibitor, on the nucleoside transport in the lung cancer cell line A549. Pharmacology 60: 34-40, 2000.
82.Matsui T, Fujimura Y, Titani K. Snake venom proteases affecting hemostasis and thrombosis. Biochim Biphys Acta (Review) 1477: 146-156, 2000.

83.Ueda S, Nishio K, Minamino N, Kubo A, Akai Y, Kanagawa K, Matsuo H, Fujimura Y, Yoshioka A, Masui K, Doi N, Murao Y, Miyamoto S. Increased plasma levels of adrenomedulin in patients with systemic inflammatory response syndrome. Am J Resp Crit Care Med 160: 132-136, 1999.
84.Tatsunami S, Mimaya J, Fukutake K, Taki M, Shirahata A, Fujimura Y, Takamatsu J, Kuwabara R, Asahara M, Yamada K. Cumulative fraction of patients with AIDS among Japanese hemophiliacs infected with HIV-1. J AIDS Research 1: 9-14, 1999.
85.Matsumoto M, Sakurai Y, Kokubo T, Yagi H, Matsui T, Titani K, Fujimura Y, Narita N. The cDNA cloning of human placental ecto-ATP diphosphohydrolases I and II. FEBS Letters 453: 335-340, 1999.
86.Matsui T, Shimoyama T, Matsumoto M, Fujimura Y, Takemoto Y, Sako M, Hamako J, Titani K. ABO blood group antigens on human von Willebrand factor after ABO-mismatched bone marrow transplantation. Blood 94: 2895-2900, 1999.
87.Miura S, Sakurai Y, Takatsuka H, Yoshioka A, Matsumoto M, Yagi H, Kokubo T, Ikeda Y, Matsui T, Titani K, Fujimura Y. Total inhibition of high shear stress induced platelet aggregation by homodimeric von Willebrand factor A1-loop fragments. Br J Haematol 105: 1092-1100, 1999.
88.Shibata M, Shima M, Fujimura Y, Takahashi Y, Nakai H, Sakurai Y, Asatani M, Nomura A, Take H, Giddings JC, Yoshioka A. Identification of the binding site for an alloantibody to von Willebrand factor which inhibits binding to glycoprotein Ib within the amino-terminal region flnaking the A1 domain. Thromb Haemost 81: 793-798, 1999.

89.Minowa R, Miyagawa S, Fukumoto T, Majima T, Shimoyama T, Fujimura Y, Shirai T. Primary Sjo¨gren's syndrome followed by chronic myelogenous leukemia: a case report with a ten year history. J Dermatology 25: 460-464, 1998.
90.Nomura S, Shouzu A, Omoto S, Hayakawa T, Kagawa H, Nishikawa M, Inaba M, Fujimurta Y, Ikeda Y, Fukuhara S. Effect of cilostazol on soluble adhesion molecules and platelet-derived microparticles in patients with diabetes. Thromb Haemost 80: 388-392, 1998.
91.Hamako J, Matsui H, Nishida S, Nomura S, Fujimura Y, Ito M, Ozeki Y, Titani K. Purification and characterization of kaouthiagin, a von Willebrand factor-binding and -cleaving metalloprotease from Naja kaouthia cobra venom. Thromb Haemost 80: 499-505, 1998.
92.Makita K, Shimoyama T, Sakurai Y, Yagi H, Matsumoto M, Narita N, Sakamoto Y, Saito S, Ikeda Y, Suzuki M, Titani K, Fujimura Y. Placental ecto-ATP diphosphohydrolase: its structural feature distinct from CD39, localization, and inhibition on shear-induced platelet aggregation. Int J Haematol 68: 297-310, 1998.
93.Tanaka Y, Hosoi G, Ishii T, Shimoyama T, Fujimura Y, Sako M. Successful engraftment of unrelated cord blood stem cell transplantation for familial erythrophagocytic lymphohistiocytosis. Bone Marrow Transplant 22: 511-513, 1998.
94.Sakurai Y, Fujimura Y, Kokubo T, Imamura K, Kawasaki T, Handa M, Suzuki M, Matsui T, Titani K, Yoshioka A. The cDNA cloning and molecular characterization of a snake venom platelet glycoprotein Ib-binding protein. Thromb Haemost 79: 1199-1207, 1998.
95.Matsui T, Hamako J, Suzuki M, Hayashi N, Ito M, Makita K, Fujimura Y, Ozeki Y, Titani K. Complete amino acid sequence of bitiscetin, a novel von Willebrand factor modulator protein, purified from snake venom of Bitis arietans. Res Commun Biochem Cell& Mol Biol 1: 217-284, 1998.

96.Matsui T, Sakurai Y, Fujimura Y, Hayashi I, Oh-ishi S, Suzuki M, Hamako J, Yamazaki J, Kinoshita M, Titani K. Purification and complete amino acid sequence of halystase from snake venom of Agkistrodon halys blomhoffi, a serine protease specifically cleaving fibrinogen and kininogen. Eur J Biochem 252: 569-575, 1997.
97.Asazuma N, Ozaki Y, Satoh K, Yatomi Y, Handa M, Fujimura Y, Miura S, Kume S. Glycoprotein Ib-von Willebrand factor interactions activate tyrosine kinases in human platelets. Blood 90: 4789-4798, 1997.
98.Yokoyama K, Handa M, Oda A, Katayama M, Fujimura Y, Murata M, Kawai Y, Watanabe K, Ikeda Y. haracterization of the novel murine monoclonal anri-von Willebrand factor (vWF) antibody GUR76-23 which inhibits vWF function with αIIbβ3 but not αvβ3 integrin. Biochem Biophys Res Commun 234: 147-152, 1997.
99.Iijima K, Murata M, Nakamura K, Kitaguchi T, Handa M, Watanabe K, Fujimura Y, Yoshioka A, Ikeda Y. High shear stress attenuates agonist-induced, glycoprotein IIb/IIIa-mediated platelet aggregation when von Willebrand factor binding to glycoprotein Ib/IX is blocked. Biochem Biophys Res Commun 233: 796-800, 1997.
100.Miyake T, Nomura S, Komiyama Y, Miyazaki Y, Kagawa H, Kitaba C, Masuda M, Fujimura Y, Ikeda Y, Fukuhara S. Effect of a new monoclonal anti-glycoprotein \ antibody, KMP-9, on high shear-induced platelet aggregation. Thromb Haemost 78: 902-909, 1997.
101.Yamanaka T, Fujimura Y, Ishimoto S, Yoshioka A, Konishi M, Narita N, Mimaya J, Meguro T, Nakasone T, Okamoto Y, Yoshizaki H, Yamada K, Honda M. Correlation of titer of antibody to principal neutralizaing domain of HIVMN strain with disease progression in Japanese hemophiliacs seropositive for HIV type 1. AIDS Res Human Retrovir 13: 317-326, 1997.
102.Matsui T, Kunishima S, Hamako J, Katayama M, Kamiya T, Naoe T, Ozeki Y, Fujimura Y, Titani K. Interaction of von Willebrand Factor with the extracellular matrix and glycocalicin under static conditions. J Biochem 121: 376-381, 1997.

103.Hamako J, Matsui T, Suzuki M, Ito M, Makita K, Fujimura Y, Ozeki Y, Titani K. Purification and characterization of bitiscetin, a novel von Willebrand factor modulator protein from Bitis arietans snake venom. Biochem Biophys Res Commun 226: 273-279, 1996.
104.Tsuji S, Sugimoto M, Kuwahara M, Nishio K, Takahashi Y, Fujimura Y, Ikeda Y, Yoshioka A. Role and initiation mechanism of the interaction of glycoprotein Ib with surface-immobilized von Willebranbd factor in a solid-phase platelet cohesion process. Blood 88: 3854-3861, 1996.
105.Miyazaki Y, Nomura S, Miyake T, Kagawa H, Kitada C, Taniguchi H, Komiyama Y, Fujimura Y, Ikeda Y, Fukuhara S. High shear stress can initiate both platelet aggregation and shedding of procoagulant containing microparticles. Blood 89:3456-3464, 1996.
106.Fujimura Y, Ishimoto S, Shimoyama T, Narita N, Kuze Y, Yoshioka A, Fukui H, Tanaka T, Tsuda F, Okamoto H, Miyakawa Y, Mayumi M. Genotypes and multiple infections with hepatitis C virus in patients with haemophilia A in Japan. J Viral Hepatitis 3: 79-84, 1996.
107.Kawasaki T, Fujimura Y, Usami Y, Suzuki M, Miura S, Sakurai Y, Makita K, Taniuchi Y, Hirano K, Titani K. Complete amino acid sequence and identification of the platelet GPIb binding site of jararaca GPIb-BP, a snake venom protein isolated from Bothrops jararaca. J Biol Chem 271: 10635-10639, 1996.
108.Usami Y, Suzuki M, Yoshida E, Sakurai Y, Hirano K, Kawasaki T, Fujimura Y, Titani K. Primary structure of alboaggregin-B purified from the venom of Trimeresurus alborablis.. Biochem Biophys Res Commun 219: 727-733, 1996.
109.Miura S, Nishida S, Makita K, Sakurai Y, Shimoyama T, Sugimoto M, Yoshioka A, Ishii K, Kito M, Kobayashi T, Fujimura Y. Inhibition assay for the binding of biotinylated von Willebrand factor to platelet-bound microtiter wells in the presence of ristocetin or botrocetin. Analyt Biochemistry 236: 215-220, 1996.
110.Fujimura Y, Kawasaki T, Titani K. Snake venom proteins modulating the interaction between von Willebrand factor and platelet glycoprotein Ib. Thromb Haemost (Review) 76: 633-639, 1996.

111.Mohri H, Tanabe J, Ohtsuka M, Yoshida M, Motomura S, Nishida S, Fujimura Y, Okubo T. Acquired von Willebrand disease associated with multiple myeloma; characterization of an inhibitor to von Willebrand factor. Blood Coagul Fibrinolys 6: 561-566, 1995.
112.Nakagawa-Nishimura Y, Sugimoto M, Tsuji S, Kawahara M, Nishio K, Takahashi Y, Fujimura Y, Murata M, Handa M, Ikeda Y, Yoshioka A. Shear stress-induced platelet aggregation in various types of von Willebland disease. Int J Haematol 66: 189-196, 1995.
113.Fujimura Y, Ikeda Y,Miura S, Yoshida E, Shima H, Nishida S, Suzuki M, Titani K, Taniuti Y, Kawasaki T. Isolation and characterization of jararaca GPIb-BP, a snake venom antagonist specific to platelet glycoprotein Ib. Thromb Haemost 74: 743-750, 1995.
114.Taniuchi Y, Kawasaki T, Fujimura Y, Suzuki M, Titani K, Sakai Y, Kaku S, Hisamichi N, Satoh N, Takenaka T, Handa M, Sawai Y. Flavocetin-A and-B, two high molecular mass glycoprotein Ib binding proteins with high affinity purified from Trimeresulus flavoviridis venom,inhibit platelet aggregation at high shear stress. Biochem Biophys Acta 1244: 331-338, 1995.
115.Kawasaki T, Taniuchi Y, Hisamichi N, Fujimura Y, Suzuki M, Koiti Titani, Kaku S, Satoh N, Takenaka T, Handa M, Sawai Y. Tokaracetin, a new platelet antagonist that binds to platelet glycoprotein Ib and inhibits von Willebrand factor-depenedent shear-induced platelet aggregation. Biochem J (GB) 308: 947-953, 1995.
116.Yoshida E, Fujimura Y, Ikeda Y, Takeda I,Yamamoto Y, Nishikawa K, Miyataka K, Oonuki M, Kawasaki T, Katayama, Narita N. Impaired high shear stress-induced platelet aggregation in patients with chronic renal failure undergoing haemodialysis. Br J Haemat 89: 861-867, 1995.
117.Shima M, Fujimura Y, Nishiyama T, Tsujiuti T, Narita N, Matsui T, Titani K, Katayama M, Yamamoto F, Yoshioka A. ABO blood group genotype and plasma von Willebland factor in nomal individuals. Vox Sanguinis 68: 236-240, 1995.
118.Katayama M, Nagata S, Hirai S, Miura S, Fujimura Y, Matsui T, Kato I, Titani K. Fibronectin and 130-kDa molecule complex mimics snake venom botrocetin-like structure potentially modulating association between von Willebland factor and vascular vessel wall. J Biochem 117: 331-338, 1995.
119. Ozaki Y, Satoh K, Yatomi Y, Miura S, Fujimura Y, Kume S. Protein tyrosine phosphorylation in human platelets induced by interaction between glycoprotein Ib and von Willebrand factor. Biochem Biophys Acta 1243: 482-488, 1995.

120. Sanma S, Momose H, Ozono S, Hirao Y, Okajima E, Morii T, Nishikawa K, Shimoyama T, Fujimura Y. Sequential changes of stem cell markers in peripheral blood and leukapheresis samples after injections of recombinant human granulocyte clony stimulating factor in patients with urogenital malignant solid tumor. Japan J Clin Oncol 24: 269-274, 1994.
121. Usami Y, Fujimura Y, Miura S, Shima H, Yoshida E, Yoshioka A, Hirano K, Suzuki M, Titani K. A 28 kDa-protein with disintegrin-like structure (jararhagin-C) purified from Bothrops jararaca venom inhibits collagen- and ADP-induced platelet aggregation. Biochem Biophys Res Commun 201: 331-339, 1994.
122. Kawasaki T, Kaku S, Kohihana T, Sakai Y, Taniuchi Y, Kawamura K, Yano S, Takenaka T, Fujimura Y. Inhibition by aurintricarboxylic acid of von Willebrand factor binding to platelet GPIb, platelet retension and thrombus formation in vitro. Am J Hematol 47: 6-15, 1994.
123. Miura S, Fujimura Y, Sugimoto M, Kawasaki T, Ikeda Y, Titani K, Yoshioka A. Structural elements influencing von Willebrand factor (vWF) binding affinity for platelet glycoprotein Ib within a dispase-digested. Blood 84: 1553-1558, 1994.
124. Nishida S, Fujimura Y, Miura M, Yoshida E, Sugimoto M, Yoshioka A, Fukui H, Ozaki Y, Usami Y, Suzuki M, Titani K. Purification and characterization of bothrombin, a fibrinogen- clotting serine protease from the venom of Bothrops jararaca. Biochemistry USA 33: 1843-1849, 1994.
125. Ozeki Y, Matsui T, Yoshida E, Fujimura Y, Nishida S, Hamako J, Usami Y, Titani K. C- type galactoside binding lectin from Bothrops jararaca venom: comparative studies of structure and function with botrocetin. Arch Biochem Biophys 308: 306-310, 1994.
126. Fujimura Y, Titani K. Structure and function of von Willebrand factor. In: Haemostais and Thrombosis (ed by Bloom AL, Forbes CD, Thomas DP, Tuddenham EGD). Churchill- Livingstone (Edinburgh, London, Madrid, Melbourne, New York, Tokyo), pp379-395, 1994.

127. Murata M, Fukuyama M, Satoh K, Fujimura Y, Yoshioka A, Takahashi H, Handa M, Kawai Y, Watanabe K, Ikeda Y. Low shear stress can initiate von Willebrand factor-dependent platelet aggregation in patients with type IIB and platelet-type von Willebrand disease. J Clin Invest 92: 1555-1558, 1993.
128. Yoshida E, Fujimura Y, Miura S, Sugimoto M, Fukui H, Narita N, Usami Y, Suzuki M, Titani K. Alboaggregin-B and botrocetin, two snake venom proteins, with highly homologous amino acid sequences but totally distinct functions on von Willebrand factor binding to platelets. Biochem Biophys Res Commun 191: 1386-1392, 1993.
129. Matsui T, Fujimura Y, Nishida S, Titani K. Human plasma α2-macroglobulin and von Willebrand factor possess covalently-linked ABO (H) blood group antigens in subjects with corresponding ABO phenotype. Blood 82: 663-668, 1993.
130. Yamamoto F, McNeil PD, Kominato K, Yamamoto M, Hakomori S, Ishimoto S, Nishida S, Shima M, Fujimura Y. Molecular genetic analysis of the ABO group system: (II) cis-AB alleles. Vox Sanguinis 64: 120-123, 1993.
131. Usami Y, Fujimura Y, Suzuki M, Ozeki Y, Nishio K, Fukui H, Titani K. Primary structure of two-chain botrocetin; a von Willebrand factor modulator purified from the venom of Bothrops jararaca. Proc Natl Acad Sci USA 90: 928-932, 1993.

132. Goto S, Ikeda Y, Murata M, Handa M, Takahashi E, Yoshioka A, Fujimura Y, Fukuyama M, Handa S, Ogawa S. Epinephrine augments von Willebrand factor dependent shear-induced platelet aggregation. Circulation 86: 1859-1863, 1992.
133. Fujimura Y, Miyata S, Nishida S, Miura S, Kaneda M, Yoshioka, A, Fukui H, Katayama M, Tuddenham EGD, Usami Y, Titani K. The interaction of botrocetin with normal or variant von Willebrand factor (types IIA and IIB) and its inhibition by monoclonal antibodies that block receptor binding. Thromb Haemost 68: 464-469, 1992.

134. Nishio K, Fujimura Y, Nishida S, Takeda I, Yoshioka A, Fukui H, Tomiyama Y, Kurata Y. Anti-platelet glycoprotein Ib monoclonal antibody (OP-F1) totally abolishes ristocetin-induced von Willebrand factor binding, but has minimal effect on the botrocetin-induced binding. Haemostasis 21: 353-359, 1991.
135. Mizumoto Y, Fujimura Y, Nishikawa K, Uchida M, Fukui H, Morii T, Narita N, Kurata Y. Flow cytometric analysis of platelet antibodies specific to the surface membrane components in patients with chronic ITP using acid treated, formalin-fixed platelets. Am J Hematol 37: 274-276, 1991.
136. Matsui T, Kihara C, Fujimura Y, Mizuoti T, Titani K. Carbohydrate analysis of human von Willebrand factor with horseradish peroxidase-conjugated lectins. Biochem Biophys Res Commun 178: 1253-1259, 1991.
137. Fujimura Y, Titani K, Usami Y, Oyama R, Matsui T, Fukui H, Sugimoto M, Ruggeri RM. Isolation and chemical characterization of two structurally and functionally distinct forms of botrocetin, the platelet coaggulutinin isolated from the venom of Bothrops jararaca. Biochemistry USA 30: 1957-1964, 1991.
138. Fujimura Y, Usami Y, Titani K, Niinomi K, Nishio K, Takase T, Yoshioka A, Fukui H. Studies on anti-von Willebrand factor (vWF) monoclonal antibody NMC-4 which inhibits both ristocetin- and botrocetin-induced vWF binding to platelet glycoprotein Ib. Blood 77: 113-120, 1991.

139. Taira T, Kamitsuji H, Okajima C, Fujimura Y. Enhanced ristocetin-induced von Willebrand factor binding to platelet glycoprotein Ib in patients with steroid-responsive nephrotic synd-rome. Haemostasis 20: 219-228, 1990.
140. Nishio K, Fujimura Y, Niinomi K, Takahashi Y, Yoshioka A, Fukui H, Usami Y, Titani T, Ruggeri TS, Zimmerman TS.Enhanced botrocetin-induced type IIB von Willebrand factor bindingto platelet glycoprotein Ib initiates hyperagglutination of normal platelets. Am J Haematol 33: 261-266, 1990.

141. Sakai T, Yoshioka A, Yamamoto Y, Niinomi K, Fujimura Y, Fukui H, Miyata T, Iwanaga S. Blood clotting factor IX Kashihara: amino acid substitution of valine-182 by phenylalanine. J Biochem 105: 756-759, 1989.
142. Saba HI, Fujimura Y, Saba SR, Bruce AC, Morelli GA,Ruggeri ZM, Zimmerman TS. Spontaneous platelet aggregation in type IIB Tampa von Willebrand disease is inhibited by the 52/48-kDa fragment of normal von Willebrand factor, which contains the GPIb binding domain. Am J Hematol 30: 150-153, 1989.
143. Adelman B, Sobel M, Fujimura Y, Ruggeri ZM, Zimmerman TS. Heparin-associated thrombocytopenia: observations on the mechanism of platelet aggregation. J Lab Clin Med 113: 204-210, 1989.
144. Fujimura Y, Ruggeri ZM, Zimmerman TS. Structure and function of human von Willebrand factor. In: Coagulation and Bleeding Disorders (ed. by Zimmerman TS and Ruggeri ZM). MarceL Dekker Inc (New York and Basel), pp77-97, 1989.

145. Mohri H, Fujimura Y, Shima M, Yoshioka A, Houghten RA, Ruggeri ZM, Zimmerman TS. Structure of the von Willebrand factor domain interacting with glycoprotein Ib. J Biol Chem 263: 17901-17904, 1988.

146. Fujimura Y, Holland LZ, Ruggeri ZM, Zimmerman TS. The von Willebrand factor domain mediating botrocetin-induced binding to glycoprotein Ib lies between Val449 and Lys728.Blood 70: 985-988, 1987.
147. Fujimura Y, Titani K, Holland LZ, Roberts JR, Kostel P, Ruggeri ZM, Zimmerman TS. A heparin-binding domain of human von Willebrand factor. Characterization and localization to a tryptic fragment extending from amino acid residue Val-449 to Lys-728. J Biol Chem 262: 1734-1739, 1987.
148. Berkowitz SD, Dent JA, Roberts JR, Fujimura Y, Plow EF, Titani K, Ruggeri ZM, Zimmerman TS. Epitope mapping of the von Willebrand factor subunit distinguishes fragments present in normal and type IIA von Willebrand disease from those generated by plasma in. J Clin Invest 79: 524-531, 1987.

149. Pareti FI, Fujimura Y, Dent JA, Holland LZ, Zimmerman TS, Ruggeri ZM. Isolation and characterization of a collagen binding domain in human von Willebrand factor. J Biol Chem 261: 1510-15315, 1986.
150. Fujimura Y, Titani K, Holland LZ, Russell SR, Roberts JR, Elder JH, Ruggeri ZM, Zimmerman TS. von Willebrand factor. A reduced and alkylated 52/48-kDa fragment beginning at amino acid residue 449 contains the domain interacting with platelet glycoprotein Ib. J Biol Chem 261: 381-385, 1986.

151. Yoshioka A, Giddings JC, Thomas JE, Fujimura Y, Bloom AL. Immunoassays of factor IX antigen using monoclonal antibodies. Br J Haematol 59: 265-275, 1985.
152. Fukui H, Fujimura Y, Sugimoto M, Okubo Y, Yoshioka A. Separation of an active substance responsible for the factor VIII-inhibitor bypassing activity in Autoplex. Acta Haematol Jap 48: 136-146, 1985.

153. Fujimura Y, Okubo Y, Sakai T, Sugimoto M, Takase T, Yoshioka A, Fukui H. Studies on precursor proteins PIVKA-II, -IX and −X in the plasma of patients with 'Hemorrhagic disease of the newborn'. Haemostasis 14: 211-217, 1984.
154. Fujimura Y, Nakaue N, Sugimoto M, Matsuyama I, Takase T,Yoshioka A, Fukui H. Congenital hypoprothrombinemia: the first reported case in Japan. Acta Paed Jap 26: 554-560, 1984.
155. Sugimoto M, Fujimura Y, Okubo Y, Takase T, Yoshioka A, Fukui H. Purification of human coagulation factor X and the immunochemical studies of factor X antigen in patients with congenital or acquired factor X deficiency. J Nara Med Assoc 35: 138-148, 1984.
156. Fujimura Y, Sugimoto M, Niinomi K, Okubo Y, Takahashi Y, Yoshioka A, Fukui H. Removal of a calcaneus pseudotumor in a factor VIII deficient patient with inhibitor using Autoplex. J Nara Med Assoc 35: 138-148,1984.
157. Takase T, Yoshikawa N, Okamoto H, Yasui M, Fujimura Y, Fukui H, Kato K. Enzyme immunoassay of α1- plasmin inhibitor. Acta Haematol Jap 47: 158-163, 1984.

158. Yoshioka A, Mikami S, Fujimura Y, Sakai T, Fukui H. Haemophilia B− in a girl. Clin Lab Haematol 4: 405-410, 1982.
159. Yoshioka K, Kinoshita S, Takamiya O, Fujimura Y, Mimura Y. Abnormal antigens of factors II, VII and IX in newborns and breast-fed infants with vitamin K deficiency. Acta Haematol Jap 45: 196-202, 1982.
160. Fujimura Y, Sakai T, Matsuyama I, Mikami S, Yoshioka A, Fukui H. A simultaneous purification of human prothrombin and factor IX. Blood Vessel 13 : 63-71,1982.
161. Fujimura Y, Mimura Y, Kinoshita S, Yoshioka A, Kitawaki T, Yoshioka K, Takamiya O. Studies on vitamin K-dependent factor deficiency during early childhood with special reference to prothrombin activity and antigen level. Haemostasis 11: 90-95, 1982.

162. Fukui H, Fujimura Y, Takase T, Shima M, Sakai T, Mikami M, Yoshioka A. Partial proteolytic susceptibility to contact activation in hemophilia B+ with slightly prolonged bovine brain prothrombin time. J Nara Med Assoc 32: 526-536, 1981.
163. Fukui H, Fujimura Y, Takahashi Y, Mikami S, Yoshioka A. Laboratory evidence of DIC under FEIBA treatment of a hemophilic patient with intracranial bleeding and high-titer factor VIII inhibitor. Thromb Res 22: 177-184, 1981.

164. Fukui H, Mikami S, Takase T, Fujimura Y, Nishino M, Yoshioka A. Patterns of factor-VIII related antigen on crossed immunoelectrophoresis and large pore polyacrylamide gel-crossed immunoelectophoresisin von Willebrand's disease. Br J Haematol 46: 269-276, 1980.

165. Fujimura Y. Studies on factor VIII procoagulant activity (VIII:C): its purification and some biochemical properties. J Nara Med Assoc 30: 199-212, 1979.
166. Fukui H, Yoshioka A, Mikami S, Takase T, Fujimura Y, Takahashi Y, Nishino M, Iwagaki K. Pathophysiology of von Willebrand's disease: qualitative and quantitative analysis of von Willebrand protein. Acta Hematol Jap 41: 839-850, 1979.

167. Yoshioka A, Fujimura Y, Yoshioka K. A note on the carrier detection of Hemophilia A. Jap Human Genet 22: 261-270, 1977.