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Literature for peptidase M12.241: ADAMTS13 peptidase

Summary Alignment Tree Sequences Sequence features Distribution Structure Literature Substrates

(Topics flags: S Structure, A Assay, P Specificity, K Knockout, E Expression, V Review, M Mutation, I Inhibitor, U Therapeutic. To select only the references relevant to a single topic, click the link above. See explanation.)

    2025
  1. Abu El-Asrar,A.M., Nawaz,M.I., Ahmad,A., Siddiquei,M., Allegaert,E., Adyns,L., Vanbrabant,L., Gikandi,P.W., De Hertogh,G., Struyf,S. and Opdenakker,G.
    ADAMTS13 Improves Endothelial Function and Reduces Inflammation in Diabetic Retinopathy
    Cells14, PubMed  Europe PubMed DOI
  2. Stanley,M., Killeen,R.B. and Michalski,J.M.
    Thrombotic Thrombocytopenic Purpura
     PubMed  Europe PubMed
  3. Stanley,M., Killeen,R.B. and Michalski,J.M.
    Thrombotic Thrombocytopenic Purpura
     PubMed  Europe PubMed
    4. 2024
  4. Bendapudi,P.K., Foy,B.H., Mueller,S.B., Liu,J., Feingold,L.M., Burke,K.E., Cruz,W., Chen,M.Y., Lau,E.S., Goldberg,R.L., Tatake,I., Wilkinson,S.C., Carney,B.J., Stone,J.R., Park,D., Avelino,A.R., Hassan,S., Andrzejewski,C., Ruby,K.N., Friedman,K.D., Brunker,P.A.R., Leaf,R.K., Higgins,J., Dzik,W.H., Stefely,J.A. and Makar,R.S.
    Recombinant ADAMTS13 for Immune Thrombotic Thrombocytopenic Purpura
    N Engl J Med390, 1690-1698. PubMed  Europe PubMed DOI
  5. Dutta Gupta,S. and Ta,M.
    ADAMTS13 regulates angiogenic markers via Ephrin/Eph signaling in human mesenchymal stem cells under serum-deprivation stress
    Sci Rep14, 560-560. PubMed  Europe PubMed DOI  K
  6. Hubben,A. and McCrae,K.R.
    A "backup plan" for ADAMTS13 deficiency in TTP
    Blood143, 2021-2023. PubMed  Europe PubMed DOI
  7. Madarati,H., Singh,K., Sparring,T., Andrisani,P., Liaw,P.C., Fox-Robichaud,A.E. and Kretz,C.A.
    REVIEWING THE DYSREGULATION OF ADAMTS13 AND VWF IN SEPSIS
    Shock61, 189-196. PubMed  Europe PubMed DOI  V
  8. Otmani,H.E., Vanhoorelbeke,K. and Tersteeg,C.
    Improving our understanding on the clinical role of plasmin-mediated von Willebrand factor degradation
    Curr Opin Hematol31, 245-250. PubMed  Europe PubMed DOI  V
  9. Papakonstantinou,A., Kalmoukos,P., Mpalaska,A., Koravou,E.E. and Gavriilaki,E.
    ADAMTS13 in the New Era of TTP
    Int J Mol Sci25, PubMed  Europe PubMed DOI
  10. Wendt,R., Volker,L., Bommer,M., Wolf,M., von Auer,C., Kuhne,L., Brinkkotter,P., Miesbach,W. and Knobl,P.
    [100 years thrombotic thrombocytopenic purpura (TTP) - lessons learned?]
    Dtsch Med Wochenschr149, 1423-1430. PubMed  Europe PubMed DOI
  11. Zhao,S.C., Li,H., Wang,M., Zhao,Y.H., Li,X.J. and Jin,S.Y.
    [ADAMTS13-Mediated Proteolytic Cleavage of Unusually Large von Willebrand Factor Polymers on Endothelial Cells in the Absence of Fluid Shear Stress]
    Zhongguo Shi Yan Xue Ye Xue Za Zhi32, 532-540. PubMed  Europe PubMed DOI
    12. 2023
  12. Barton,J.C., Anderson,C., Miranda,F.Z., Kelley,R., Kremer Hovinga,J.A., Terrell,D., Vesely,S.K., George,J.N. and Muia,J.
    Cattle-FRETS71, a novel fluorogenic substrate with broad applicability for characterizing ADAMTS13 properties and function
    J Thromb Haemost21, 3393-3401. PubMed  Europe PubMed DOI
  13. Beckman,J.D. and Sparkenbaugh,E.M.
    The invisible string of coagulation, complement, iron, and inflammation in sickle cell disease
    Curr Opin Hematol30, 153-158. PubMed  Europe PubMed DOI  V
  14. Dekimpe,C., Roose,E., Sakai,K., Tersteeg,C., De Meyer,S.F. and Vanhoorelbeke,K.
    Toward gene therapy for congenital thrombotic thrombocytopenic purpura
    J Thromb Haemost21, 1090-1099. PubMed  Europe PubMed DOI  V
  15. Gao,D., Zhou,Z., Ma,R., Wu,H., Nguyen,T., Liu,L. and Dong,J.
    Recombinant ADAMTS-13 Improves Survival of Mice Subjected to Endotoxemia
    Int J Mol Sci24, PubMed  Europe PubMed DOI
  16. Lammle,B.
    Mechanisms of ADAMTS13 inhibition in iTTP
    Blood141, 2919-2920. PubMed  Europe PubMed DOI
  17. Lin,J., Ding,X., Yang,P., Liu,S., Li,Q., Cruz,M.A., Dong,J.F., Fang,Y. and Wu,J.
    Force-induced biphasic regulation of VWF cleavage by ADAMTS13
    Thromb Res229, 99-106. PubMed  Europe PubMed DOI
  18. Rossato,P., Glantschnig,H., Canneva,F., Schuster,M., Coulibaly,S., Schrenk,G., Voelkel,D., Dockal,M., Plaimauer,B., Rottensteiner,H., Gritsch,H., Federti,E., Matte,A., De Franceschi,L., Scheiflinger,F. and Hoellriegl,W.
    Treatment with recombinant ADAMTS13, alleviates hypoxia/reoxygenation-induced pathologies in a mouse model of human sickle cell disease
    J Thromb Haemost21, 269-275. PubMed  Europe PubMed DOI
  19. Wang,X., Hao,X.J., Dai,C.G., Ding,Y.J., Xiong,L., Deng,J. and Jiang,J.J.
    Identification of 8 Rare Deleterious Variants in ADAMTS13 by Next-generation Sequencing in a Chinese Population with Thrombotic Thrombocytopenic Purpura
    Curr Med Sci43, 1043-1050. PubMed  Europe PubMed DOI
    20. 2022
  20. Chaturvedi,S. and DeBaun,M.R.
    Altered VWF:ADAMTS13 homeostasis is a target for therapeutic intervention in sickle cell disease
    Proc Natl Acad Sci U S A119, e2213079119-e2213079119. PubMed  Europe PubMed DOI
  21. DeYoung,V., Singh,K. and Kretz,C.A.
    Mechanisms of ADAMTS13 regulation
    J Thromb Haemost20, 2722-2732. PubMed  Europe PubMed DOI  V
  22. Fujimura,Y. and Holland,L.Z.
    COVID-19 microthrombosis: unusually large VWF multimers are a platform for activation of the alternative complement pathway under cytokine storm
    Int J Hematol115, 457-469. PubMed  Europe PubMed DOI
  23. Jankowska,K.I., Meyer,D., Holcomb,D.D., Kames,J., Hamasaki-Katagiri,N., Katneni,U.K., Hunt,R.C., Ibla,J.C. and Kimchi-Sarfaty,C.
    Synonymous ADAMTS13 variants impact molecular characteristics and contribute to variability in active protein abundance
    Blood Adv6, 5364-5378. PubMed  Europe PubMed DOI
  24. Liu,W. and Xiao,Y.
    [Advance in the diagnosis and treatment of hereditary thrombotic thrombocytopenic purpura]
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi39, 442-446. PubMed  Europe PubMed DOI  V
  25. Merlen,C., Pepin,E., Barry,O., Cormier,A., Dubois,C., Lapeyraque,A.L., Troyanov,S., Rivard,G.E. and Bonnefoy,A.
    Incidence of thrombotic microangiopathies in Quebec: insight from a laboratory centralizing ADAMTS-13 testing
    Orphanet J Rare Dis17, 308-308. PubMed  Europe PubMed DOI
    26. 2021
  26. Beranger,N., Benghezal,S., Joly,B.S., Capdenat,S., Delton,A., Stepanian,A., Coppo,P. and Veyradier,A.
    Diagnosis and follow-up of thrombotic thrombocytopenic purpura with an automated chemiluminescent ADAMTS13 activity immunoassay
    Res Pract Thromb Haemost5, 81-93. PubMed  Europe PubMed DOI
  27. Favaloro,E.J., Pasalic,L., Henry,B. and Lippi,G.
    Laboratory testing for ADAMTS13: Utility for TTP diagnosis/exclusion and beyond
    Am J Hematol96, 1049-1055. PubMed  Europe PubMed DOI  A  V
  28. Kamenac,A., Obser,T., Wixforth,A., Schneider,M.F. and Westerhausen,C.
    The activity of the intrinsically water-soluble enzyme ADAMTS13 correlates with the membrane state when bound to a phospholipid bilayer
    Sci Rep11, 24476-24476. PubMed  Europe PubMed DOI
  29. Miodownik,S., Pikovsky,O., Erez,O., Kezerle,Y., Lavon,O. and Rabinovich,A.
    Unfolding the pathophysiology of congenital thrombotic thrombocytopenic purpura in pregnancy: Lessons from a cluster of familial cases
    Am J Obstet Gynecol225, 177-177. PubMed  Europe PubMed DOI
  30. Popa,M., Hecker,M. and Wagner,A.H.
    Inverse Regulation of Confluence-Dependent ADAMTS13 and von Willebrand Factor Expression in Human Endothelial Cells
    Thromb Haemost122, 611-622. PubMed  Europe PubMed DOI
  31. Reardon,B., Pasalic,L. and Favaloro,E.J.
    The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease
    J Cardiovasc Dev Dis8, PubMed  Europe PubMed DOI  V
  32. Turecek,P.L., Peck,R.C., Rangarajan,S., Reilly-Stitt,C., Laffan,M.A., Kazmi,R., James,I., Dushianthan,A., Schrenk,G., Gritsch,H., Ewenstein,B.M., Mellgard,B., Erdlenbruch,W., Jain,N., Binder,N.B. and Mumford,A.D.
    Recombinant ADAMTS13 reduces abnormally up-regulated von Willebrand factor in plasma from patients with severe COVID-19
    Thromb Res201, 100-112. PubMed  Europe PubMed DOI
  33. Wu,H., Li,H., Su,C., Li,H.Y., Cui,R.H. and Jin,S.Y.
    [Biological Function of CysR Domain of ADAMTS13]
    Zhongguo Shi Yan Xue Ye Xue Za Zhi29, 893-900. PubMed  Europe PubMed DOI
  34. Yoshitaka,M.
    [Thrombotic thrombocytopenic purpura]
    Rinsho Ketsueki62, 430-434. PubMed  Europe PubMed DOI  V
    35. 2020
  35. Al-Awadhi,A., Adekile,A. and Marouf,R.
    Relationship of Thrombospondin 1 to von Willebrand Factor and ADAMTS-13 in Sickle Cell Disease Patients of Arab Ethnicity
    Acta Haematol144, 1-8. PubMed  Europe PubMed DOI
  36. Jacquemin,M., Van Horenbeeck,I., Debasse,M., Toelen,J., Schoeters,J., Vanlinthout,I., Peerlinck,K., Dierickx,D. and Van Laer,C.
    Optimization of the detection of inhibitory autoantibodies against the VWF-cleaving protease ADAMTS13 with an automated chemiluminescent ADAMTS13 activity immunoassay
    Int J Lab Hematol43, 290-297. PubMed  Europe PubMed DOI
  37. Letzer,A., Lehmann,K., Mess,C., Konig,G., Obser,T., Peine,S., Schneppenheim,S., Budde,U., Schneider,S.W., Schneppenheim,R. and Brehm,M.A.
    Upshaw-Schulman syndrome-associated ADAMTS13 variants possess proteolytic activity at the surface of endothelial cells and in simulated circulation
    PLoS ONE15, e0232637-e0232637. PubMed  Europe PubMed DOI  M
  38. Moller,C., Schutte,A.E., Smith,W. and Botha-Le Roux,S.
    Von Willebrand factor, its cleaving protease (ADAMTS13), and inflammation in young adults: The African-PREDICT study
    Cytokine136, 155265-155265. PubMed  Europe PubMed DOI
  39. Stratmann,J., Ward,J.N. and Miesbach,W.
    Evaluation of a rapid turn-over, fully-automated ADAMTS13 activity assay: a method comparison study
    J Thromb Thrombolysis50, 628-631. PubMed  Europe PubMed DOI  A
    40. 2019
  40. Hunt,R., Hettiarachchi,G., Katneni,U., Hernandez,N., Holcomb,D., Kames,J., Alnifaidy,R., Lin,B., Hamasaki-Katagiri,N., Wesley,A., Kafri,T., Morris,C., Bouche,L., Panico,M., Schiller,T., Ibla,J., Bar,H., Ismail,A., Morris,H., Komar,A. and Kimchi-Sarfaty,C.
    A single synonymous variant (c.354G>A [p.P118P]) in ADAMTS13 confers enhanced specific activity
    Int J Mol Sci20, 5734-5734. PubMed  Europe PubMed DOI  M
  41. Ma,Z.N., Ling,J., Yin,J., Shen,F., Su,J., Xie,L.Q. and Ruan,C.G.
    [Effect of recombinant protein in the spacer domain on ADAMTS13 activity]
    Zhongguo Shi Yan Xue Ye Xue Za Zhi27, 1596-1601. PubMed  Europe PubMed DOI  U
  42. Rottensteiner,H., Seyfried,B.K., Kaufmann,S., Fiedler,C., Dong,J.F., Zheng,X.L., Plaimauer,B. and Scheiflinger,F.
    Identification of cysteine thiol-based linkages in ADAMTS13 in support of a non-proteolytic regulation of von Willebrand factor
    J Thromb Haemost17, 2099-2109. PubMed  Europe PubMed DOI
    43. 2018
  43. Abdelgawwad,M.S., Cao,W., Zheng,L., Kocher,N.K., Williams,L.A. and Zheng,X.L.
    Transfusion of platelets loaded with recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) is efficacious for inhibiting arterial thrombosis associated with thrombotic thrombocytopenic purpura
    Arterioscler Thromb Vasc Biol38, 2731-2743. PubMed  Europe PubMed DOI  U
  44. Clark,C.C., Mebius,M.M., de Maat,S., Tielens,A.G.M., DE Groot,P.G., Urbanus,R.T., Fijnheer,R., Hazenberg,B.P.C., van Hellemond,J.J. and Maas,C.
    Truncation of ADAMTS13 by plasmin enhances its activity in plasma
    Thromb Haemost118, 471-479. PubMed  Europe PubMed DOI
  45. Ercig,B., Wichapong,K., Reutelingsperger,C.P.M., Vanhoorelbeke,K., Voorberg,J. and Nicolaes,G.A.F.
    Insights into 3D structure of ADAMTS13: a stepping stone towards novel therapeutic treatment of thrombotic thrombocytopenic purpura
    Thromb Haemost118, 28-41. PubMed  Europe PubMed DOI  S
  46. Romao de Souza V Jr, Beatriz Cavalcante de Oliveira,A., Maria Vanderlei,A., Queiroz da Mota Silveira Aroucha,A., Pontes Duarte,B., Nunes Machado,A., Netto Chaer,L., Wanderley de Barros Correia,C., da Conceicao de Barros Correia,M. and Freire Hazin Costa,M.
    Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report
    J Med Case Rep12, 15-15. PubMed  Europe PubMed DOI  M
    47. 2017
  47. Aponte-Santamaria,C., Lippok,S., Mittag,J.J., Obser,T., Schneppenheim,R., Baldauf,C., Grater,F., Budde,U. and Radler,J.O.
    Mutation G1629E increases von Willebrand factor cleavage via a cooperative destabilization mechanism
    Biophys J112, 57-65. PubMed  Europe PubMed DOI
  48. Cao,W.J. and Zheng,X.L.
    Conformational quiescence of ADAMTS-13 prevents proteolytic promiscuity: comment
    J Thromb Haemost15, 586-589. PubMed  Europe PubMed DOI
  49. Garland,K.S., Reitsma,S.E., Shirai,T., Zilberman-Rudenko,J., Tucker,E.I., Gailani,D., Gruber,A., McCarty,O.J.T. and Puy,C.
    Removal of the C-terminal domains of ADAMTS13 by activated coagulation factor XI induces platelet adhesion on endothelial cells under flow conditions
    Front Med (Lausanne)4, 232-232. PubMed  Europe PubMed DOI
  50. Gogia,S., Kelkar,A., Zhang,C., Dayananda,K.M. and Neelamegham,S.
    Role of calcium in regulating the intra- and extracellular cleavage of von Willebrand factor by the protease ADAMTS13
    Blood Adv1, 2063-2074. PubMed  Europe PubMed DOI
  51. Lynch,C.J., Cawte,A.D., Millar,C.M., Rueda,D. and Lane,D.A.
    A common mechanism by which type 2A von Willebrand disease mutations enhance ADAMTS13 proteolysis revealed with a von Willebrand factor A2 domain FRET construct
    PLoS ONE12, e0188405-e0188405. PubMed  Europe PubMed DOI
  52. South,K., Freitas,M.O. and Lane,D.A.
    A model for the conformational activation of the structurally quiescent metalloprotease ADAMTS13 by Von Willebrand factor
    J Biol Chem292, 5760-5769. PubMed  Europe PubMed DOI
  53. South,K., Freitas,M.O. and Lane,D.A.
    Conformational quiescence of ADAMTS-13 prevents proteolytic promiscuity: reply
    J Thromb Haemost15, 589-590. PubMed  Europe PubMed DOI
  54. Verhenne,S., Vandeputte,N., Pareyn,I., Izsvak,Z., Rottensteiner,H., Deckmyn,H., De Meyer,S.F. and Vanhoorelbeke,K.
    Long-term prevention of congenital thrombotic thrombocytopenic purpura in ADAMTS13 knockout mice by sleeping beauty transposon-mediated gene therapy
    Arterioscler Thromb Vasc Biol37, 836-844. PubMed  Europe PubMed DOI  U
    55. 2016
  55. Erpenbeck,L., Demers,M., Zsengeller,Z.K., Gallant,M., Cifuni,S.M., Stillman,I.E., Karumanchi,S.A. and Wagner,D.D.
    ADAMTS13 endopeptidase protects against vascular endothelial growth factor inhibitor-induced thrombotic microangiopathy
    J Am Soc Nephrol27, 120-131. PubMed  Europe PubMed DOI  U
  56. Guo,C., Tsigkou,A. and Lee,M.H.
    ADAMTS13 and 15 are not regulated by the full length and N-terminal domain forms of TIMP-1, -2, -3 and -4
    Biomed Rep4, 73-78. PubMed  Europe PubMed DOI  I
  57. Ostertag,E.M., Kacir,S., Thiboutot,M., Gulendran,G., Zheng,X.L., Cines,D.B. and Siegel,D.L.
    ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 1. Structural and functional characterization in vitro
    Transfusion56, 1763-1774. PubMed  Europe PubMed DOI
  58. South,K., Freitas,M.O. and Lane,D.A.
    Conformational quiescence of ADAMTS-13 prevents proteolytic promiscuity
    J Thromb Haemost14, 2011-2022. PubMed  Europe PubMed DOI
  59. Tersteeg,C., Fijnheer,R., Pasterkamp,G., DE Groot,P.G., Vanhoorelbeke,K., de Maat,S. and Maas,C.
    Keeping von Willebrand factor under control: alternatives for ADAMTS13
    Semin Thromb Hemost42, 9-17. PubMed  Europe PubMed DOI  M
    60. 2015
  60. de Groot,R., Lane,D.A. and Crawley,J.T.
    The role of the ADAMTS13 cysteine-rich domain in VWF binding and proteolysis
    Blood125, 1968-1975. PubMed  Europe PubMed DOI
  61. Deforche,L., Roose,E., Vandenbulcke,A., Vandeputte,N., Feys,H.B., Springer,T.A., Mi,L.Z., Muia,J., Sadler,J.E., Soejima,K., Rottensteiner,H., Deckmyn,H., De Meyer,S.F. and Vanhoorelbeke,K.
    Linker regions and flexibility around the metalloprotease domain account for conformational activation of ADAMTS-13
    J Thromb Haemost13, 2063-2075. PubMed  Europe PubMed DOI
  62. Desch,K.C., Kretz,C., Yee,A., Gildersleeve,R., Metzger,K., Agrawal,N., Cheng,J. and Ginsburg,D.
    Probing ADAMTS13 substrate specificity using phage display
    PLoS ONE10, e0122931- PubMed  Europe PubMed
  63. Dubail,J. and Apte,S.S.
    Insights on ADAMTS proteases and ADAMTS-like proteins from mammalian genetics
    Matrix Biol44-46, 24-37. PubMed  Europe PubMed DOI
  64. Kretz,C.A., Dai,M., Soylemez,O., Yee,A., Desch,K.C., Siemieniak,D., Tomberg,K., Kondrashov,F.A., Meng,F. and Ginsburg,D.
    Massively parallel enzyme kinetics reveals the substrate recognition landscape of the metalloprotease ADAMTS13
    Proc Natl Acad Sci U S A112, 9328-9333. PubMed  Europe PubMed DOI  P
  65. Lu,R.N., Yang,S., Wu,H.M. and Zheng,X.L.
    Unconjugated bilirubin inhibits proteolytic cleavage of von Willebrand factor by ADAMTS13 protease
    J Thromb Haemost13, 1064-1072. PubMed  Europe PubMed DOI  I
  66. Plaimauer,B., Schiviz,A., Kaufmann,S., Hollriegl,W., Rottensteiner,H. and Scheiflinger,F.
    Neutralization of inhibitory antibodies and restoration of therapeutic ADAMTS-13 activity levels in inhibitor-treated rats by the use of defined doses of recombinant ADAMTS-13
    J Thromb Haemost13, 2053-2062. PubMed  Europe PubMed DOI
  67. Wang,Y., Chen,J., Ling,M., Lopez,J.A., Chung,D.W. and Fu,X.
    Hypochlorous acid generated by neutrophils inactivates ADAMTS13: an oxidative mechanism for regulating ADAMTS13 proteolytic activity during inflammation
    J Biol Chem290, 1422-1431. PubMed  Europe PubMed DOI
    68. 2014
  68. Mancini,I., Valsecchi,C., Lotta,L.A., Deforche,L., Pontiggia,S., Bajetta,M., Palla,R., Vanhoorelbeke,K. and Peyvandi,F.
    FRETS-VWF73 rather than CBA assay reflects ADAMTS13 proteolytic activity in acquired thrombotic thrombocytopenic purpura patients
    Thromb Haemost112, 297-303. PubMed  Europe PubMed DOI  A
  69. Perez-Rodriguez,A., Loures,E., Rodriguez-Trillo,A., Costa-Pinto,J., Garcia-Rivero,A., Batlle-Lopez,A., Batlle,J. and Lopez-Fernandez,M.F.
    Inherited ADAMTS13 deficiency (Upshaw-Schulman syndrome): a short review
    Thromb Res134, 1171-1175. PubMed  Europe PubMed DOI  M
  70. Wang,Q. and Han,Y.
    [Structure and function of ADAMTS13 protease and its relation with diagnosis and treatment of TTP]
    Zhongguo Shi Yan Xue Ye Xue Za Zhi22, 1157-1161. PubMed  Europe PubMed DOI  V
    71. 2013
  71. Ling,J., Su,J., Ma,Z. and Ruan,C.
    The WXXW motif in the TSR1 of ADAMTS13 is important for its secretion and proteolytic activity
    Thromb Res131, 529-534. PubMed  Europe PubMed DOI
  72. Ling,J., Ma,Z.N., Su,J. and Ruan,C.G.
    [Construction of ADAMTS13-pEGFP-N1 vector and its expression in HeLa cells]
    Zhongguo Shi Yan Xue Ye Xue Za Zhi21, 126-129. PubMed  Europe PubMed DOI  U
  73. Lotta,L.A., Wu,H.M., Musallam,K.M. and Peyvandi,F.
    The emerging concept of residual ADAMTS13 activity in ADAMTS13-deficient thrombotic thrombocytopenic purpura
    Blood Rev27, 71-76. PubMed  Europe PubMed DOI  V
  74. Zheng,X.L.
    Structure-function and regulation of ADAMTS-13 protease
    J Thromb Haemost11 Suppl 1, 11-23. PubMed  Europe PubMed DOI
    75. 2012
  75. Ferrari,S., Rottensteiner,H. and Scheiflinger,F.
    ADAMTS13: von Willebrand factor-cleaving protease
    [ISSN:978-0-12-407744-7]3, 1199-1206. DOI
  76. Gao,W., Zhu,J., Westfield,L.A., Tuley,E.A., Anderson,P.J. and Sadler,J.E.
    Rearranging exosites in noncatalytic domains can redirect the substrate specificity of ADAMTS proteases
    J Biol Chem287, 26944-26952. PubMed  Europe PubMed DOI
  77. Maruyama,H., Ito,K., Okabayashi,K., Sakai,M., Kano,R., Watari,T., Hasegawa,A. and Kamata,H.
    Molecular cloning, in vitro expression and functional characterization of canine ADAMTS13
    Res Vet Sci93, 213-218. PubMed  Europe PubMed DOI  E
  78. Pozzi,N., Lancellotti,S., De Cristofaro,R. and De Filippis,V.
    Modeling ADAMTS13-von Willebrand factor interaction: implications for oxidative stress-related cardiovascular diseases and type 2A von Willebrand Disease
    Biophys Chem160, 1-11. PubMed  Europe PubMed DOI
  79. Schiviz,A., Wuersch,K., Piskernik,C., Dietrich,B., Hoellriegl,W., Rottensteiner,H., Scheiflinger,F., Schwarz,H.P. and Muchitsch,E.M.
    A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13
    Blood119, 6128-6135. PubMed  Europe PubMed DOI  U
  80. Shin,Y., Akiyama,M., Kokame,K., Soejima,K. and Miyata,T.
    Binding of von Willebrand factor cleaving protease ADAMTS13 to Lys-plasmin(ogen)
    J Biochem152, 251-258. PubMed  Europe PubMed DOI
    81. 2011
  81. Crawley,J.T., de Groot,R., Xiang,Y., Luken,B.M. and Lane,D.A.
    Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor
    Blood118, 3212-3221. PubMed  Europe PubMed DOI  P
  82. De Ceunynck,K., Rocha,S., Feys,H.B., De Meyer,S.F., Uji,I., Deckmyn,H., Hofkens,J. and Vanhoorelbeke,K.
    Local elongation of endothelial cell-anchored von Willebrand factor strings precedes ADAMTS13 protein-mediated proteolysis
    J Biol Chem286, 36361-36367. PubMed  Europe PubMed DOI
  83. Hunt,R.C., Geetha,S., Allen,C.E., Hershko,K., Fathke,R., Kong,P.L., Plum,E., Struble,E.B., Soejima,K., Friedman,S., Garfield,S., Balaji,S. and Kimchi-Sarfaty,C.
    Detection of a secreted metalloprotease within the nuclei of liver cells
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