臨床検査52巻13号

2008年12月発行

今月の主題 凝固制御 血液凝固系検査の新展開

ADAMTS13

著者： 小亀浩市¹

所属機関： ¹国立循環器病センター研究所脈管生理部

ページ範囲：P.1566 - P.1570

　ADAMTS13はvon Willebrand因子を切断することで止血機能を調節する抗血栓性因子である．ADAMTS13活性の欠損は血小板の過剰凝集を引き起こし，血栓性血小板減少性紫斑病(TTP)の発症要因となる．近年，ADAMTS13活性測定法の簡便化が進み，自己抗体の検出や抗原量の測定とともに，容易に検査できるようになった．TTPの確定診断や鑑別診断，除外診断などに役立つことが期待されている．

参考文献

1) Levy GG, Nichols WC, Lian EC, et al：Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature　413：488-494, 2001

2) Soejima K, Mimura N, Hirashima M, et al：A novel human metalloprotease synthesized in the liver and secreted into the blood：possibly, the von Willebrand factor-cleaving protease? J Biochem　130：475-480, 2001

3) Z Zheng X, Chung D, Takayama TK, et al：Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem　276：41059-41063, 2001

proteolysis. Blood　87：4223-4234, 1996

5) Tsai HM：Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood　87：4235-4244, 1996

6) Gerritsen HE, Turecek PL, Schwarz HP, et al：Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF：a tool for the diagnosis of thrombotic thrombocytopenic purpura(TTP). Thromb Haemost　82：1386-1389, 1999

7) Obert B, Tout H, Veyradier A, et al：Estimation of the von Willebrand factor-cleaving protease in plasma using monoclonal antibodies to vWF. Thromb Haemost　82：1382-1385, 1999

8) Böhm M, Vigh T, Scharrer I：Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13). Ann Hematol　81：430-435, 2002

9) Dong JF, Moake JL, Nolasco L, et al：ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood　100：4033-4039, 2002

10) Kokame K, Matsumoto M, Fujimura Y, et al：VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13. Blood　103：607-612, 2004

11) Kokame K, Nobe Y, Kokubo Y, et al：FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay. Br J Haematol　129：93-100, 2005

12) Kato S, Matsumoto M, Matsuyama T, et al：Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity. Transfusion　46：1444-1452, 2006

13) Wu JJ, Fujikawa K, Lian EC, et al：A rapid enzyme-linked assay for ADAMTS-13. J Thromb Haemost　4：129-136, 2006

14) Jin M, Cataland S, Bissell M, et al：A rapid test for the diagnosis of thrombotic thrombocytopenic purpura using surface enhanced laser desorption/ionization time-of-flight (SELDI-TOF)-mass spectrometry. J Thromb Haemost　4：333-338, 2006

15) Groot E, Hulstein JJ, Rison CN, et al：FRETS-VWF73：a rapid and predictive tool for thrombotic thrombocytopenic purpura. J Thromb Haemost　4：698-699, 2006

16) Kremer Hovinga JA, Mottini M, Lämmle B：Measurement of ADAMTS-13 activity in plasma by the FRETS-VWF73 assay：comparison with other assay methods. J Thromb Haemost　4：1146-1148, 2006

17) Mahdian R, Rayes J, Girma JP, et al：Comparison of FRETS-VWF73 to full-length VWF as a substrate for ADAMTS13 activity measurement in human plasma samples. Thromb Haemost　95：1049-1051, 2006

. J Thromb Haemost　4：1707-1717, 2006

19) Ono T, Mimuro J, Madoiwa S, et al：Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation：its correlation with development of renal failure. Blood　107：528-534, 2006

20) Rieger M, Ferrari S, Kremer Hovinga JA, et al：Relation between ADAMTS13 activity and ADAMTS13 antigen levels in healthy donors and patients with thrombotic microangiopathies (TMA). Thromb Haemost　95：212-220, 2006

21) Soejima K, Nakamura H, Hirashima M, et al：Analysis on the molecular species and concentration of circulating ADAMTS13 in Blood. J Biochem　139：147-154, 2006

22) Kokame K, Matsumoto M, Soejima K, et al：Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity. Proc Natl Acad Sci USA　99：11902-11907, 2002

. J Thromb Haemost　6：1830-1832, 2008