ADAMTS13 turns 3.

作者: Gallia G. Levy , David G. Motto , David Ginsburg

DOI: 10.1182/BLOOD-2004-10-4097

关键词: Thrombocytopenic purpuraVon Willebrand factorHemostasisImmunologyThrombotic thrombocytopenic purpuraADAMTS13ThrombospondinMedicineHematologic diseasePathogenesis

摘要: It has now been 3 years since the von Willebrand factor (VWF)–cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (adisintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 mutations resulting familial TTP have reported. Considerable progress also realized toward understanding role of normal hemostasis, well mechanisms by which deficiency contributes to pathogenesis. Measurement activity other pathologic conditions remains a focus substantial clinical research effort. Building on these studies, continued investigation VWF holds considerable promise for advancing should lead improved diagnosis treatment this important hematologic disease.

参考文章(105)
Alan F. Scott, Joanna Amberger, Brandon Brylawski, Victor A. McKusick, OMIM: Online Mendelian Inheritance in Man Bioinformatics: Databases and Systems. pp. 77- 84 ,(2002) , 10.1007/0-306-46903-0_7
Miha Furlan, Rodolfo Robles, Max Solenthaler, Max Wassmer, Pierre Sandoz, Bernhard Lämmle, Deficient Activity of von Willebrand Factor–Cleaving Protease in Chronic Relapsing Thrombotic Thrombocytopenic Purpura Blood. ,vol. 89, pp. 3097- 3103 ,(1997) , 10.1182/BLOOD.V89.9.3097
Patrick André, Cécile V. Denis, Jerry Ware, Simin Saffaripour, Richard O. Hynes, Zaverio M. Ruggeri, Denisa D. Wagner, Platelets adhere to and translocate on von Willebrand factor presented by endothelium in stimulated veins Blood. ,vol. 96, pp. 3322- 3328 ,(2000) , 10.1182/BLOOD.V96.10.3322
HM Tsai, RL Nagel, VB Hatcher, II Sussman, Multimeric composition of endothelial cell-derived von Willebrand factor. Blood. ,vol. 73, pp. 2074- 2076 ,(1989) , 10.1182/BLOOD.V73.8.2074.2074
Miha Furlan, Rodolfo Robles, Max Solenthaler, Bernhard Lämmle, Acquired Deficiency of von Willebrand Factor-Cleaving Protease in a Patient With Thrombotic Thrombocytopenic Purpura Blood. ,vol. 91, pp. 2839- 2846 ,(1998) , 10.1182/BLOOD.V91.8.2839.2839_2839_2846