ADAMTS13

Summary

Gene Symbol: ADAMTS13
Description: ADAM metallopeptidase with thrombospondin type 1 motif 13
Alias: ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP, A disintegrin and metalloproteinase with thrombospondin motifs 13, a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13, vWF-cleaving protease, von Willebrand factor-cleaving protease
Species: human
Products:     ADAMTS13

Top Publications

  1. 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. 2004;103:607-12 pubmed
    ..Thus, VWF73 will be a useful seed to develop a new rapid assay to determine ADAMTS-13 activity. ..
  2. Cao W, Krishnaswamy S, Camire R, Lenting P, Zheng X. Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13. Proc Natl Acad Sci U S A. 2008;105:7416-21 pubmed publisher
    Proteolytic processing of von Willebrand factor (VWF) by ADAMTS13 metalloproteinase is crucial for normal hemostasis...
  3. Luken B, Turenhout E, Hulstein J, van Mourik J, Fijnheer R, Voorberg J. The spacer domain of ADAMTS13 contains a major binding site for antibodies in patients with thrombotic thrombocytopenic purpura. Thromb Haemost. 2005;93:267-74 pubmed
    ..thrombocytopenic purpura (TTP) is a microangiopathy often associated with a severely decreased activity of ADAMTS13. In plasma of the majority of patients with TTP, antibodies are present that inhibit the von Willebrand factor (..
  4. Turner N, Nolasco L, Ruggeri Z, Moake J. Endothelial cell ADAMTS-13 and VWF: production, release, and VWF string cleavage. Blood. 2009;114:5102-11 pubmed publisher
    ..HUVEC ADAMTS13 mRNA expression was approximately 1:100 of VWF monomeric subunit expression...
  5. Majerus E, Anderson P, Sadler J. Binding of ADAMTS13 to von Willebrand factor. J Biol Chem. 2005;280:21773-8 pubmed
    b>ADAMTS13, a metalloprotease, cleaves von Willebrand factor (VWF) in plasma to generate smaller, less thrombogenic fragments...
  6. Chion C, Doggen C, Crawley J, Lane D, Rosendaal F. ADAMTS13 and von Willebrand factor and the risk of myocardial infarction in men. Blood. 2007;109:1998-2000 pubmed
    ..This function depends on its multimeric size, which is controlled by ADAMTS13. We measured plasma ADAMTS13 and VWF antigen levels by enzyme-linked immunosorbent assay (ELISA) in a large ..
  7. Ai J, Smith P, Wang S, Zhang P, Zheng X. The proximal carboxyl-terminal domains of ADAMTS13 determine substrate specificity and are all required for cleavage of von Willebrand factor. J Biol Chem. 2005;280:29428-34 pubmed
    b>ADAMTS13 limits platelet-rich thrombosis by cleaving von Willebrand factor at the Tyr(1605)-Met(1606) bond. Previous studies showed that ADAMTS13 truncated after spacer domain remains proteolytically active or hyperactive...
  8. Levy G, Motto D, Ginsburg D. ADAMTS13 turns 3. Blood. 2005;106:11-7 pubmed
    ..factor (VWF)-cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13)...
  9. Uchida T, Wada H, Mizutani M, Iwashita M, Ishihara H, Shibano T, et al. Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura. Blood. 2004;104:2081-3 pubmed
    ..uremic syndrome (TTP/HUS) is associated with an inherited von Willebrand factor-cleaving protease (ADAMTS13 [a disintegrin and metalloprotease with thrombospondin type I domains 13]) deficiency...

More Information

Publications65

  1. Kokame K, Miyata T. Genetic defects leading to hereditary thrombotic thrombocytopenic purpura. Semin Hematol. 2004;41:34-40 pubmed
    ..23 patients with hereditary TTP and their families has been analyzed and 33 causative mutations identified in the ADAMTS13 gene: 19 missense, five nonsense, five frameshift, and four splice mutations...
  2. Dong J. Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions. J Thromb Haemost. 2005;3:1710-6 pubmed
    ..Dissecting the process of ULVWF proteolysis is important for not only understanding the pathophysiology of thrombotic microangiopathies, but also developing more effective means to treat these deadly diseases. ..
  3. Tao Z, Peng Y, Nolasco L, Cal S, Lopez Otin C, Li R, et al. Recombinant CUB-1 domain polypeptide inhibits the cleavage of ULVWF strings by ADAMTS13 under flow conditions. Blood. 2005;106:4139-45 pubmed
    The metalloprotease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin motif) converts the hyperreactive unusually large (UL) forms of von Willebrand factor (VWF) that are newly released from endothelial cells into less ..
  4. Shomron N, Hamasaki Katagiri N, Hunt R, Hershko K, Pommier E, Geetha S, et al. A splice variant of ADAMTS13 is expressed in human hepatic stellate cells and cancerous tissues. Thromb Haemost. 2010;104:531-5 pubmed publisher
    Although ADAMTS13, the von Willebrand factor (VWF)-cleaving protease, is expressed in a range of tissues, the physiological significance of tissue-specific ADAMTS13 alternative splicing isoforms have yet to be clarified...
  5. Kokame K, Matsumoto M, Soejima K, Yagi H, Ishizashi H, Funato M, 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;99:11902-7 pubmed
    ..of the corresponding cDNA revealed that the 1,427-aa polypeptide is a member of the ADAMTS gene family, termed ADAMTS13. Twelve rare mutations in this gene have been identified in patients with congenital TTP...
  6. Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001;98:1662-6 pubmed
    ..An active site sequence of HEIGHSFGLEHE (single-letter amino acid codes) was located at 150 residues from the N terminus of the protein. ..
  7. Shim K, Anderson P, Tuley E, Wiswall E, Sadler J. Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress. Blood. 2008;111:651-7 pubmed
    Endothelial cells secrete prothrombotic ultralarge von Willebrand factor (VWF) multimers, and the metalloprotease ADAMTS13 cleaves them into smaller, less dangerous multimers...
  8. Plaimauer B, Fuhrmann J, Mohr G, Wernhart W, Bruno K, Ferrari S, et al. Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation. Blood. 2006;107:118-25 pubmed
    Sequence analysis of the ADAMTS13 locus of 2 patients with hereditary thrombotic thrombocytopenic purpura (TTP) revealed the homozygous presence of 4 single nucleotide polymorphisms (SNPs) (R7W, Q448E, P618A, A732V) and a rare missense ..
  9. Sauna Z, Okunji C, Hunt R, Gupta T, Allen C, Plum E, et al. Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease. PLoS ONE. 2009;4:e6506 pubmed publisher
    The zinc metalloprotease ADAMTS13 is a multidomain protein that cleaves von Willebrand Factor (VWF) and is implicated in Thrombotic Thrombocytopenic Purpura (TTP) pathogenesis...
  10. Powazniak Y, Kempfer A, Pereyra J, Palomino J, Lazzari M. VWF and ADAMTS13 behavior in estradiol-treated HUVEC. Eur J Haematol. 2011;86:140-7 pubmed publisher
    In this study, the role of 17?-estradiol (E2) in the regulation of von Willebrand factor (VWF) and ADAMTS13 synthesis, storage, and secretion was investigated in cultured human umbilical vein endothelial cells (HUVEC)...
  11. Bianchi V, Robles R, Alberio L, Furlan M, Lammle B. Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood. 2002;100:710-3 pubmed
    A severe deficiency in von Willebrand factor-cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a ..
  12. Hassenpflug W, Budde U, Obser T, Angerhaus D, Drewke E, Schneppenheim S, et al. Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis. Blood. 2006;107:2339-45 pubmed
    ..Underlying mutations cluster in the A2 domain of VWF around its cleavage site for ADAMTS13. We investigated the impact of mutations commonly found in patients with VWD type 2A on ADAMTS13-dependent ..
  13. Rieger M, Mannucci P, Kremer Hovinga J, Herzog A, Gerstenbauer G, Konetschny C, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood. 2005;106:1262-7 pubmed
    Autoantibodies neutralizing human ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif), the metalloprotease that physiologically cleaves von Willebrand factor, are a major cause of severe deficiency of the ..
  14. Zanardelli S, Chion A, Groot E, Lenting P, McKinnon T, Laffan M, et al. A novel binding site for ADAMTS13 constitutively exposed on the surface of globular VWF. Blood. 2009;114:2819-28 pubmed publisher
    b>ADAMTS13 metalloprotease regulates the multimeric size of von Willebrand factor (VWF) by cleaving the Tyr1605-Met1606 bond in the VWF A2 domain. The mechanisms of VWF recognition by ADAMTS13 have yet to be fully resolved...
  15. Vomund A, Majerus E. ADAMTS13 bound to endothelial cells exhibits enhanced cleavage of von Willebrand factor. J Biol Chem. 2009;284:30925-32 pubmed publisher
    b>ADAMTS13 is a plasma metalloprotease that cleaves ultralarge von Willebrand factor multimers to generate less thrombogenic fragments...
  16. Rayes J, Hommais A, Legendre P, Tout H, Veyradier A, Obert B, et al. Effect of von Willebrand disease type 2B and type 2M mutations on the susceptibility of von Willebrand factor to ADAMTS-13. J Thromb Haemost. 2007;5:321-8 pubmed
    ..In VWD type 2B, the spontaneous binding to platelets and excessive degradation by ADAMTS-13 of VWF high-molecular-weight multimers may account for their clearance from plasma. ..
  17. Jang M, Kim N, Chong S, Kim H, Lee S, Kang M, et al. Frequency of Pro475Ser polymorphism of ADAMTS13 gene and its association with ADAMTS-13 activity in the Korean population. Yonsei Med J. 2008;49:405-8 pubmed publisher
    ..The frequency of the C1423T allele of the ADAMTS13 gene was studied along with measuring plasma ADAMTS-13 activity in 250 healthy Korean individuals...
  18. Gerritsen H, Robles R, Lammle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood. 2001;98:1654-61 pubmed
    ..There was even a temporary increase in protease activity during the first 3 days of incubation. ..
  19. Löwenberg E, Charunwatthana P, Cohen S, van den Born B, Meijers J, Yunus E, et al. Severe malaria is associated with a deficiency of von Willebrand factor cleaving protease, ADAMTS13. Thromb Haemost. 2010;103:181-7 pubmed publisher
    ..disease strongly associated with a deficiency of the von Willebrand factor (VWF) cleaving protease, ADAMTS13. We measured plasma ADAMTS13 activity, VWF antigen and VWF propeptide levels in 30 patients with severe ..
  20. Plaimauer B, Zimmermann K, Völkel D, Antoine G, Kerschbaumer R, Jenab P, et al. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood. 2002;100:3626-32 pubmed
    ..gene encoding for VWF-cp has recently been identified as a member of the metalloprotease family and designated ADAMTS13, but the functional activity of the ADAMTS13 gene product has not been verified...
  21. Dong J, Moake J, Bernardo A, Fujikawa K, Ball C, Nolasco L, et al. ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor. J Biol Chem. 2003;278:29633-9 pubmed
    ..Once released, these cleaved VWF fragments become inaccessible for the metalloprotease to prevent further cleavage. ..
  22. Bongers T, De Maat M, Dippel D, Uitterlinden A, Leebeek F. Absence of Pro475Ser polymorphism in ADAMTS-13 in Caucasians. J Thromb Haemost. 2005;3:805 pubmed
  23. Shang D, Zheng X, Niiya M, Zheng X. 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;108:2207-15 pubmed
    b>ADAMTS13 biosynthesis appeared to occur mainly in hepatic stellate cells, but detection of ADAMTS13 mRNA in many other tissues suggests that vascular endothelium may also produce ADAMTS13...
  24. Soejima K, Mimura N, Hirashima M, Maeda H, Hamamoto T, Nakagaki T, 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;130:475-80 pubmed
  25. Levy G, Nichols W, Lian E, Foroud T, McClintick J, McGee B, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413:488-94 pubmed
    ..of a much larger transcript, identifying a new member of the ADAMTS family of zinc metalloproteinase genes (ADAMTS13)...
  26. Soejima K, Matsumoto M, Kokame K, Yagi H, Ishizashi H, Maeda H, et al. ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage. Blood. 2003;102:3232-7 pubmed
    ..The major epitopes of these antibodies were found to reside within the cysteine-rich/spacer domains. These results suggest that the ADAMTS-13 cysteine-rich/spacer domains are essential for VWF-CP activity. ..
  27. Dai L, Su J, Wang Z, Ruan C. The frequency of P475S polymorphism in von Willebrand factor-cleaving protease in the Chinese population and its relevance to arterial thrombotic disorders. Thromb Haemost. 2004;91:1257-8 pubmed
  28. Skipwith C, Cao W, Zheng X. Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress. J Biol Chem. 2010;285:28596-603 pubmed publisher
    ..have demonstrated that factor VIII (FVIII) or platelets alone increase cleavage of von Willebrand factor (VWF) by ADAMTS13 under mechanically induced shear stresses...
  29. Raife T, Cao W, Atkinson B, Bedell B, Montgomery R, Lentz S, et al. Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site. Blood. 2009;114:1666-74 pubmed publisher
    ..The importance of ADAMTS13 metalloprotease in VWF regulation is demonstrated by the association between severe deficiency of ADAMTS13 and ..
  30. Kraisin S, Naka I, Patarapotikul J, Nantakomol D, Nuchnoi P, Hananantachai H, et al. Association of ADAMTS13 polymorphism with cerebral malaria. Malar J. 2011;10:366 pubmed publisher
    ..studies reported increased levels of von Willebrand factor (VWF) and reduced activity of VWF-cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), in patients with cerebral ..
  31. De Meyer S, Savchenko A, HAAS M, Schatzberg D, Carroll M, Schiviz A, et al. Protective anti-inflammatory effect of ADAMTS13 on myocardial ischemia/reperfusion injury in mice. Blood. 2012;120:5217-23 pubmed publisher
    ..To investigate a potential protective role of ADAMTS13, a protease cleaving von Willebrand factor multimers, during myocardial ischemia/reperfusion, we used a mouse ..
  32. Crawley J, Lane D, Woodward M, Rumley A, Lowe G. Evidence that high von Willebrand factor and low ADAMTS-13 levels independently increase the risk of a non-fatal heart attack. J Thromb Haemost. 2008;6:583-8 pubmed publisher
    ..The association of ADAMTS-13 with MI is influenced by lipid levels, and consequently requires further investigation. ..
  33. Furlan M, Robles R, Lammle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87:4223-34 pubmed
    ..The size, amino acid composition, and amino terminal sequence of the reduced fragments confirmed that the peptide bond 842Tyr-843Met had been cleaved, ie, the same bond that has been proposed to be cleaved in vivo. ..
  34. Shibagaki Y, Matsumoto M, Kokame K, Ohba S, Miyata T, Fujimura Y, et al. 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. 2006;21:1289-92 pubmed
    ..patients with Upshaw-Schulman syndrome (USS)--a congenital deficiency of von Willebrand factor-cleaving protease (ADAMTS13) activity--responds very well to plasma infusion and does not even require plasma exchange...
  35. Shida Y, Nishio K, Sugimoto M, Mizuno T, Hamada M, Kato S, et al. Functional imaging of shear-dependent activity of ADAMTS13 in regulating mural thrombus growth under whole blood flow conditions. Blood. 2008;111:1295-8 pubmed
    The metalloprotease ADAMTS13 is assumed to regulate the functional levels of von Willebrand factor (VWF) appropriate for normal hemostasis in vivo by reducing VWF multimer size, which directly represents the thrombogenic activity of this ..
  36. Scheiflinger F, Knobl P, Trattner B, Plaimauer B, Mohr G, Dockal M, et al. Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura. Blood. 2003;102:3241-3 pubmed
    ..Given that ADAMTS-13 may interact physiologically with various receptors or ligands, the occurrence, distribution, and the epitope mapping of nonneutralizing antibodies will be an important area for future research. ..
  37. Taniguchi S, Hashiguchi T, Ono T, Takenouchi K, Nakayama K, Kawano T, et al. Association between reduced ADAMTS13 and diabetic nephropathy. Thromb Res. 2010;125:e310-6 pubmed publisher
    Deficiency of Von Willebrand factor (VWF)-cleaving protease (ADAMTS13) causes platelet thrombosis in the microcirculation. Intrarenal coagulation is thought to be associated with the development and progression of diabetic nephropathy...
  38. Tsai H. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996;87:4235-44 pubmed
    ..This model may explain the decrease in vWF multimer sizes in various clinical conditions. ..
  39. Tao Z, Anthony K, Peng Y, Choi H, Nolasco L, Rice L, et al. Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura. J Thromb Haemost. 2006;4:1931-5 pubmed
    ..Mutations throughout the ADAMTS13 gene have been identified in congenital TTP patients, most of whom have initial episodes during infancy or in ..
  40. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89:3097-103 pubmed
    ..The assay of the vWF-cleaving protease activity may be used as a sensitive diagnostic tool for identification of subjects with a latent TTP tendency. ..
  41. Zheng X, Chung D, Takayama T, Majerus E, Sadler J, Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. 2001;276:41059-63 pubmed
    ..sequence, VWFCP was identified recently as a new member of the ADAMTS family of metalloproteases and designated ADAMTS13. The 4.6-kilobase pair cDNA sequence for VWFCP has now been determined...
  42. Nishio K, Anderson P, Zheng X, Sadler J. Binding of platelet glycoprotein Ibalpha to von Willebrand factor domain A1 stimulates the cleavage of the adjacent domain A2 by ADAMTS13. Proc Natl Acad Sci U S A. 2004;101:10578-83 pubmed
    ..Domain A2 contains the Tyr-1605-Met-1606 bond that is cleaved by the metalloprotease ADAMTS13, and this reaction inhibits platelet thrombus growth...
  43. Dong J, Moake J, Nolasco L, Bernardo A, Arceneaux W, Shrimpton C, et al. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood. 2002;100:4033-9 pubmed
    ..These results suggest that cleavage of EC-derived ULVWF multimers by ADAMTS-13 is a rapid physiologic process that occurs on endothelial cell surfaces. ..
  44. Schneppenheim R, Budde U, Oyen F, Angerhaus D, Aumann V, Drewke E, et al. von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood. 2003;101:1845-50 pubmed
    ..multimers of von Willebrand factor (VWF) due to deficiency of the specific VWF-cleaving protease (VWF-CP) ADAMTS13, resulting in microangiopathic disease...
  45. Majerus E, Zheng X, Tuley E, Sadler J. Cleavage of the ADAMTS13 propeptide is not required for protease activity. J Biol Chem. 2003;278:46643-8 pubmed
    b>ADAMTS13 belongs to the "a disintegrin and metalloprotease with thrombospondin repeats" family, and cleaves von Willebrand factor multimers into smaller forms...
  46. Liu F, Jin J, Dong N, Wang Y, Ruan C. [Identification of two novel mutations in ADAMTS13 gene in a patient with hereditary thrombotic thrombocytopenic purpura]. Zhonghua Xue Ye Xue Za Zhi. 2005;26:521-4 pubmed
    To investigate the gene mutations of ADAMTS13 in a highly suspected hereditary thrombocytopenic purpura (TTP) patient, and then make a progressive diagnosis and adjust the plan of therapy...
  47. Veyradier A, Lavergne J, Ribba A, Obert B, Loirat C, Meyer D, et al. Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome). J Thromb Haemost. 2004;2:424-9 pubmed
    b>ADAMTS13, the specific von Willebrand factor (VWF)-cleaving metalloprotease, prevents the spontaneous formation of platelet thrombi in the microcirculation by degrading the highly adhesive ultralarge VWF multimers into smaller forms...
  48. Lotta L, Garagiola I, Palla R, Cairo A, Peyvandi F. ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura. Hum Mutat. 2010;31:11-9 pubmed publisher
    ..microangiopathic hemolytic anemia, associated with the deficiency of the von Willebrand factor-cleaving protease (ADAMTS13) due to mutations in the corresponding gene...
  49. Uemura M, Tatsumi K, Matsumoto M, Fujimoto M, Matsuyama T, Ishikawa M, et al. Localization of ADAMTS13 to the stellate cells of human liver. Blood. 2005;106:922-4 pubmed
    Although the chromosomal localization (9q34) of the gene encoding the human form of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) and its exclusive expression in the liver have been established, ..
  50. Zhou W, Dong L, Ginsburg D, Bouhassira E, Tsai H. Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: a novel therapeutic strategy?. J Biol Chem. 2005;280:39934-41 pubmed
    b>ADAMTS13 (a disintegrin and metalloprotease with thrombospondin motifs), a circulating multidomain zinc metalloprotease of the reprolysin subfamily, is critical for preventing von Willebrand factor-platelet interaction under high shear ..
  51. Wang Y, Fan W, Zhao S, Zhang K, Zhang L, Zhang P, et al. Qualitative, quantitative and combination score systems in differential diagnosis of breast lesions by contrast-enhanced ultrasound. Eur J Radiol. 2016;85:48-54 pubmed publisher
    ..The application of qualitative could develop the diagnostic performance of CEUS which is clinically promising. ..
  52. Khaw A, Angermaier A, Michel P, Kirsch M, Kessler C, Langner S. Inter-rater Agreement in Three Perfusion-Computed Tomography Evaluation Methods before Endovascular Therapy for Acute Ischemic Stroke. J Stroke Cerebrovasc Dis. 2016;25:960-8 pubmed publisher
    ..QPRs in PCT evaluation had better inter-rater reliability than the often used VME and ASPECTS-PCT assessment. ..
  53. Di Costanzo G, Tortora R, Morisco F, Addario L, Guarino M, Cordone G, et al. Impact of Diabetes on Outcomes of Sorafenib Therapy for Hepatocellular Carcinoma. Target Oncol. 2017;12:61-67 pubmed publisher
    ..038). Sorafenib was as safe as effective in DIAB and in nDIAB patients. The longer TTP observed among DIAB than in nDIAB patients might suggest a better anticancer effect of sorafenib in patients with diabetes. ..
  54. Gao W, Wang Z, Bai X, Ruan C. [Study of C1423T polymorphism of the von Willebrand factor-cleaving protease gene in Chinese Han population]. Zhonghua Xue Ye Xue Za Zhi. 2004;25:136-8 pubmed
    ..No 1423T/T homozygote was found. Besides, there was no significant difference between healthy controls and patients with thrombotic disorders. C1423T polymorphism is low frequency in both controls and patients of Han population. ..
  55. Chinen Y, Kuroda J, Ohshiro M, Shimura Y, Mizutani S, Nagoshi H, et al. Low ADAMTS-13 activity during hemorrhagic events with disseminated intravascular coagulation. Int J Hematol. 2013;97:511-9 pubmed publisher
    ..007). Plasma ADAMTS-13 activity determined at diagnosis of DIC may help predict the risk of hemorrhagic events during and/or following DIC treatment with hematologic disorders. ..
  56. Qiu T, Wang H, Song J, Ling W, Shi Y, Guo G, et al. Assessment of liver fibrosis by ultrasound elastography and contrast-enhanced ultrasound: a randomized prospective animal study. Exp Anim. 2017;: pubmed publisher
    ..845 vs. 0.956 (P=0.04), 0.789 vs. 0.954 (P=0.01) and 0.707 vs. 0.933 (P=0.03). Both CEUS and pSWE had the potential to be complementary imaging tools in the evaluation of liver fibrosis. The performance of pSWE may be better than CEUS...