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血管性血友病因子裂解酶的研究进展 被引量：1

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摘要 大量研究表明，在生理条件下，血浆中血管性血友病因子（von Willebrand factor，VWF）主要受金属蛋白酶，即血管性血友病因子裂解酶（von Willebrand factor—cleaving protease，VWF-cp）的调节，VWF—cp活性异常致血浆中超大VwF多聚体（UL．VwF）无法降解并蓄积，是导致血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura，TTP）发病的重要机制。
作者 曹文静 ZHENG X Long
机构地区 华中科技大学同济医学院附属同济医院检验科 美国宾夕法尼亚大学费城儿童医院病理系
出处 《中华检验医学杂志》 CAS CSCD 北大核心 2008年第6期704-707,共4页 Chinese Journal of Laboratory Medicine
关键词 血管性血友病因子裂解酶 Willebrand 血栓性血小板减少性紫癜 PROTEASE 金属蛋白酶 多聚体 VWF VWF
分类号 R686 [医药卫生—骨科学]
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参考文献37

  • 1Fujikawa K, Suzuki H, McMullen B, et al. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood, 2001, 98: 1662-1666.
  • 2Gerritsen HE, Robles R, Lammle B, et al. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood, 2001, 98:1654-1661.
  • 3Soejima 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, 2001, 130:475-480.
  • 4Zheng X, Chung D, Takayama TK, et al. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metallopwtease involved in thrombotic thrombocytopenic purpura. J Biol Chem, 2001,276:41059-41063.
  • 5Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature, 2001, 413:488-494.
  • 6Kokame 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 U S A, 2002, 99:11902-11907.
  • 7Majerus EM, Zheng X, Tuley EA, et al. Cleavage of the ADAMTS13 propeptide is not required for protease activity. J Biol Chem, 2003, 278:46643-46648.
  • 8Shang D, Zheng XW, Niiya M, et al. Apical sorting of ADAMTS13 in vascular endothelial cells and Madin-Darby canine kidney cells depends on the CUB domains and their association with lipid rafts. Blood, 2006, 108:2207-2215.
  • 9Zheng X, Nishio K, Majerus EM, et al. Cleavage of yon Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13. J Biol Chem, 2003, 278:30136-30141.
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同被引文献14

  • 1王学文.血栓性血小板减少性紫癜患者血浆VW因子裂解蛋白酶活性测定的临床意义[J].医学研究生学报,2005,18(3):250-254. 被引量：3
  • 2李世军,陈惠萍.血栓性微血管病的分类及发病机制[J].肾脏病与透析肾移植杂志,2006,15(6):546-551. 被引量：2
  • 3韩红,邹萍.血栓性血小板减少性紫癜及其研究进展[J].临床内科杂志,2007,24(2):142-144. 被引量：22
  • 4Moskowitz NP,Fligman I,Scimeea P,et al.Transient TTP in Childhood[J].Pediatr Blood Cancer,2009,52(3):424-426.
  • 5Rock GA,Shurnak KH,Buskard NA,et al.Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura Canadian Aphcresis Study Group[J].N Engl J Meal,1991,325(6):393-397.
  • 6Sarode R.Atypical Presentations of Thrombotic Thrombocytopenic Purpura:A Review[J].Journal of Clinical Apheresis,2009,24(1):47-52.
  • 7Tao Z,Anthony K,Peng Y,et al.Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura[J].J Thromb Hacmost,2006,4(9):1931-1935.
  • 8Shibagaki Y,Toshiro F.Thrombotic Microangiopathy in Malignant Hypertension and Hemolytic Uremic Syndrome(HUS)/Thrombotic Thrombocytopenic Purpura(TTP):Can We Differentiate One from the Other?[J].Hypertens Res,2005,28(1):89-95.
  • 9Caramello V,Dovio A,Caraci P,et al.Thrombotic thrombocytopenic purpttra in advanced prostate cancer:Case report and published work review[J].International Journal of Urology,2007,14(2):150-152.
  • 10Rock GA,Shumak KH,Buskard NA,et al.Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura.Canadian Apheresis Study Group[J].N Engl J Med,1991,325(6):393-397.

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中华检验医学杂志
2008年 第6期

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