SEARCH:   Advanced

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Gerritsen, H. E. Articles by Furlan, M. Search for Related Content PubMed PubMed Citation Articles by Gerritsen, H. E. Articles by Furlan, M. Related Collections Red Cells Plenary Papers Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 September 2001, Vol. 98, No. 6, pp. 1654-1661 PLENARY PAPER Partial amino acid sequence of purified von Willebrand factor-cleaving protease Helena E. Gerritsen, Rodolfo Robles, Bernhard Lämmle, and Miha Furlan From the Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland. von Willebrand factor-cleaving protease (vWF-cp) is responsible for the continuous degradation of plasma vWF multimers released from endothelial cells. It is deficient in patients with thrombotic thrombocytopenic purpura, who show unusually large vWF multimers in plasma. Purified vWF-cp may be useful for replacement in these patients, who are now treated by plasma therapy. In this study, vWF-cp was purified from normal human plasma by affinity chromatography on the IgG fraction from a patient with autoantibodies to vWF-cp and by a series of further chromatographic procedures, including affinity chromatography on Protein G, Ig-TheraSorb, lentil lectin, and heparin. Four single-chain protein bands, separated by sodium dodecyl sulfate-polyacrylamide gel electrophoresis under nonreducing conditions, showed Mr of 150, 140, 130, and 110 kd and were found to share the same N-terminal amino acid sequence, suggesting that they were derived from the same polypeptide chain that had been partially degraded at the carboxy-terminal end. A hydrophobic sequence (Ala-Ala-Gly-Gly-Ile-Leu-His-Leu-Glu-Leu-Leu-Val-Ala-Val-Gly) of the first 15 residues was established. The protease migrates in gel filtration as a high-molecular-weight complex with clusterin, a 70-kd protein with chaperonelike activity. vWF-cp bound to clusterin is dissociated by the use of concentrated chaotropic salts. vWF-cp in normal human plasma or serum is not associated with clusterin, suggesting that the observed complex is due to vWF-cp denaturation during the purification procedure. Activity of vWF-cp is unusually stable during incubation at 37°C; its in vitro half-life in citrated human plasma, heparin plasma, or serum is longer than 1 week. There was even a temporary increase in protease activity during the first 3 days of incubation. © 2001 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. F. De Meyer, H. Deckmyn, and K. Vanhoorelbeke von Willebrand factor to the rescue Blood, May 21, 2009; 113(21): 5049 - 5057. [Abstract] [Full Text] [PDF] I. Garagiola, C. Valsecchi, S. Lavoretano, H. Oren, M. Bohm, and F. Peyvandi Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion Haematologica, November 1, 2008; 93(11): 1678 - 1685. [Abstract] [Full Text] [PDF] J. E. Sadler Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura Blood, July 1, 2008; 112(1): 11 - 18. [Abstract] [Full Text] [PDF] B. Lammle, J. A. Kremer Hovinga, and J. N. George Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease Haematologica, February 1, 2008; 93(2): 172 - 177. [Full Text] [PDF] F. Peyvandi, S. Lavoretano, R. Palla, H. B. Feys, K. Vanhoorelbeke, T. Battaglioli, C. Valsecchi, M. T. Canciani, F. Fabris, S. Zver, et al. ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission Haematologica, February 1, 2008; 93(2): 232 - 239. [Abstract] [Full Text] [PDF] P. J. Anderson, D. Gailani, H. B. Feys, W. Gao, E. M. Majerus, K. Vanhoorelbeke, and J. E. Sadler Factor XI/ADAMTS13 complexes are quantitatively insignificant in human plasma Haematologica, October 1, 2007; 92(10): 1419 - 1422. [Abstract] [Full Text] [PDF] K. C. Desch and D. G. Motto Thrombotic Thrombocytopenic Purpura in Humans and Mice Arterioscler Thromb Vasc Biol, September 1, 2007; 27(9): 1901 - 1908. [Abstract] [Full Text] [PDF] K. K. Swisher, J. T. Doan, S. K. Vesely, H. C. Kwaan, B. Kim, B. Lammle, J. A. Kremer Hovinga, and J. N. George Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports Haematologica, July 1, 2007; 92(7): 936 - 943. [Abstract] [Full Text] [PDF] B.J. Hunt, S. Tueger, J. Pattison, J. Cavenagh, and D.P. D'Cruz Microangiopathic haemolytic anaemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura Lupus, May 1, 2007; 16(5): 358 - 362. [Abstract] [PDF] A. Casonato, F. Fabris, E. Pontara, M. G. Cattini, N. Zocca, L. Gallinaro, A. Girolami, and A. Pagnan Diagnosis and Follow-up of Thrombotic Thrombocytopenic Purpura by Means of von Willebrand Factor Collagen Binding Assay Clinical and Applied Thrombosis/Hemostasis, July 1, 2006; 12(3): 296 - 304. [Abstract] [PDF] W. A. Hassenpflug, U. Budde, T. Obser, D. Angerhaus, E. Drewke, S. Schneppenheim, and R. Schneppenheim Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis Blood, March 15, 2006; 107(6): 2339 - 2345. [Abstract] [Full Text] [PDF] T. Ono, J. Mimuro, S. Madoiwa, K. Soejima, Y. Kashiwakura, A. Ishiwata, K. Takano, T. Ohmori, and Y. Sakata 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, January 15, 2006; 107(2): 528 - 534. [Abstract] [Full Text] [PDF] K. Soejima, H. Nakamura, M. Hirashima, W. Morikawa, C. Nozaki, and T. Nakagaki Analysis on the Molecular Species and Concentration of Circulating ADAMTS13 in Blood J. Biochem., January 1, 2006; 139(1): 147 - 154. [Abstract] [Full Text] [PDF] J. E. Sadler Thrombotic Thrombocytopenic Purpura: A Moving Target Hematology, January 1, 2006; 2006(1): 415 - 420. [Abstract] [Full Text] [PDF] B. Plaimauer, J. Fuhrmann, G. Mohr, W. Wernhart, K. Bruno, S. Ferrari, C. Konetschny, G. Antoine, M. Rieger, and F. Scheiflinger Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation Blood, January 1, 2006; 107(1): 118 - 125. [Abstract] [Full Text] [PDF] J. J. J. Hulstein, P. G. de Groot, K. Silence, A. Veyradier, R. Fijnheer, and P. J. Lenting A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B Blood, November 1, 2005; 106(9): 3035 - 3042. [Abstract] [Full Text] [PDF] J. Ai, P. Smith, S. Wang, P. Zhang, and X. L. Zheng The Proximal Carboxyl-terminal Domains of ADAMTS13 Determine Substrate Specificity and Are All Required for Cleavage of von Willebrand Factor J. Biol. Chem., August 19, 2005; 280(33): 29428 - 29434. [Abstract] [Full Text] [PDF] G. G. Levy, D. G. Motto, and D. Ginsburg ADAMTS13 turns 3 Blood, July 1, 2005; 106(1): 11 - 17. [Abstract] [Full Text] [PDF] Z. Tao, Y. Wang, H. Choi, A. Bernardo, K. Nishio, J. E. Sadler, J. A. Lopez, and J.-f. Dong Cleavage of ultralarge multimers of von Willebrand factor by C-terminal-truncated mutants of ADAMTS-13 under flow Blood, July 1, 2005; 106(1): 141 - 143. [Abstract] [Full Text] [PDF] E. M. Majerus, P. J. Anderson, and J. E. Sadler Binding of ADAMTS13 to von Willebrand Factor J. Biol. Chem., June 10, 2005; 280(23): 21773 - 21778. [Abstract] [Full Text] [PDF] J. T. B. Crawley, J. K. Lam, J. B. Rance, L. R. Mollica, J. S. O'Donnell, and D. A. Lane Proteolytic inactivation of ADAMTS13 by thrombin and plasmin Blood, February 1, 2005; 105(3): 1085 - 1093. [Abstract] [Full Text] [PDF] T. Uchida, H. Wada, M. Mizutani, M. Iwashita, H. Ishihara, T. Shibano, M. Suzuki, Y. Matsubara, K. Soejima, M. Matsumoto, et al. Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura Blood, October 1, 2004; 104(7): 2081 - 2083. [Abstract] [Full Text] [PDF] R. P. T. Somerville, J.-M. Longpre, E. D. Apel, R. M. Lewis, L. W. Wang, J. R. Sanes, R. Leduc, and S. S. Apte ADAMTS7B, the Full-length Product of the ADAMTS7 Gene, Is a Chondroitin Sulfate Proteoglycan Containing a Mucin Domain J. Biol. Chem., August 20, 2004; 279(34): 35159 - 35175. [Abstract] [Full Text] [PDF] F. Banno, K. Kaminaka, K. Soejima, K. Kokame, and T. Miyata Identification of Strain-specific Variants of Mouse Adamts13 Gene Encoding von Willebrand Factor-cleaving Protease J. Biol. Chem., July 16, 2004; 279(29): 30896 - 30903. [Abstract] [Full Text] [PDF] C. Klaus, B. Plaimauer, J.-D. Studt, F. Dorner, B. Lammle, P. M. Mannucci, and F. Scheiflinger Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura Blood, June 15, 2004; 103(12): 4514 - 4519. [Abstract] [Full Text] [PDF] X. L. Zheng, R. M. Kaufman, L. T. Goodnough, and J. E. Sadler Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura Blood, June 1, 2004; 103(11): 4043 - 4049. [Abstract] [Full Text] [PDF] J.-D. Studt, J. A. K. Hovinga, R. Radonic, V. Gasparovic, D. Ivanovic, M. Merkler, U. Wirthmueller, C. Dahinden, M. Furlan, and B. Lammle Familial acquired thrombotic thrombocytopenic purpura: ADAMTS13 inhibitory autoantibodies in identical twins Blood, June 1, 2004; 103(11): 4195 - 4197. [Abstract] [Full Text] [PDF] A. Padilla, J. L. Moake, A. Bernardo, C. Ball, Y. Wang, M. Arya, L. Nolasco, N. Turner, M. C. Berndt, B. Anvari, et al. P-selectin anchors newly released ultralarge von Willebrand factor multimers to the endothelial cell surface Blood, March 15, 2004; 103(6): 2150 - 2156. [Abstract] [Full Text] [PDF] M. Matsumoto, K. Kokame, K. Soejima, M. Miura, S. Hayashi, Y. Fujii, A. Iwai, E. Ito, Y. Tsuji, M. Takeda-Shitaka, et al. Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome Blood, February 15, 2004; 103(4): 1305 - 1310. [Abstract] [Full Text] [PDF] K. Kokame, M. Matsumoto, Y. Fujimura, and T. Miyata VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13 Blood, January 15, 2004; 103(2): 607 - 612. [Abstract] [Full Text] [PDF] E. M. Majerus, X. Zheng, E. A. Tuley, and J. E. Sadler Cleavage of the ADAMTS13 Propeptide Is Not Required for Protease Activity J. Biol. Chem., November 21, 2003; 278(47): 46643 - 46648. [Abstract] [Full Text] [PDF] K. Soejima, M. Matsumoto, K. Kokame, H. Yagi, H. Ishizashi, H. Maeda, C. Nozaki, T. Miyata, Y. Fujimura, and T. Nakagaki ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage Blood, November 1, 2003; 102(9): 3232 - 3237. [Abstract] [Full Text] [PDF] K. Varadi, J. Schreiner, B. Plaimauer, M. Rieger, F. Scheiflinger, P. Knobl, P. L. Turecek, and H. P. Schwarz ADAMTS13 autoantibody detection by quantitative immunoblotting Blood, September 1, 2003; 102(5): 1932 - 1933. [Full Text] [PDF] J.-f. Dong, J. L. Moake, A. Bernardo, K. Fujikawa, C. Ball, L. Nolasco, J. A. Lopez, and M. A. Cruz ADAMTS-13 Metalloprotease Interacts with the Endothelial Cell-derived Ultra-large von Willebrand Factor J. Biol. Chem., August 8, 2003; 278(32): 29633 - 29639. [Abstract] [Full Text] [PDF] X. Zheng, K. Nishio, E. M. Majerus, and J. E. Sadler Cleavage of von Willebrand Factor Requires the Spacer Domain of the Metalloprotease ADAMTS13 J. Biol. Chem., August 8, 2003; 278(32): 30136 - 30141. [Abstract] [Full Text] [PDF] M. Llamazares, S. Cal, V. Quesada, and C. Lopez-Otin Identification and Characterization of ADAMTS-20 Defines a Novel Subfamily of Metalloproteinases-Disintegrins with Multiple Thrombospondin-1 Repeats and a Unique GON Domain J. Biol. Chem., April 4, 2003; 278(15): 13382 - 13389. [Abstract] [Full Text] [PDF] H.-M. Tsai Advances in the Pathogenesis, Diagnosis, and Treatment of Thrombotic Thrombocytopenic Purpura J. Am. Soc. Nephrol., April 1, 2003; 14(4): 1072 - 1081. [Abstract] [Full Text] [PDF] R. Schneppenheim, U. Budde, F. Oyen, D. Angerhaus, V. Aumann, E. Drewke, W. Hassenpflug, J. Haberle, K. Kentouche, E. Kohne, et al. von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP Blood, March 1, 2003; 101(5): 1845 - 1850. [Abstract] [Full Text] [PDF] X. Zheng, A. M. Pallera, L. T. Goodnough, J. E. Sadler, and M. A. Blinder Remission of Chronic Thrombotic Thrombocytopenic Purpura after Treatment with Cyclophosphamide and Rituximab Ann Intern Med, January 21, 2003; 138(2): 105 - 108. [Abstract] [Full Text] [PDF] J.-f. Dong, J. L. Moake, L. Nolasco, A. Bernardo, W. Arceneaux, C. N. Shrimpton, A. J. Schade, L. V. McIntire, K. Fujikawa, and J. A. Lopez ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions Blood, December 1, 2002; 100(12): 4033 - 4039. [Abstract] [Full Text] [PDF] H.-M. Tsai, B. Lammle, V. Bianchi, L. Alberio, M. Furlan, G. Remuzzi, M. Galbusera, and P. M. Mannucci Deficiency of ADAMTS13 and thrombotic thrombocytopenic purpura Blood, November 15, 2002; 100(10): 3839 - 3842. [Full Text] [PDF] B. Plaimauer, K. Zimmermann, D. Volkel, G. Antoine, R. Kerschbaumer, P. Jenab, M. Furlan, H. Gerritsen, B. Lammle, H. P. Schwarz, et al. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13) Blood, November 15, 2002; 100(10): 3626 - 3632. [Abstract] [Full Text] [PDF] C. R. Flannery, W. Zeng, C. Corcoran, L. A. Collins-Racie, P. S. Chockalingam, T. Hebert, S. A. Mackie, T. McDonagh, T. K. Crawford, K. N. Tomkinson, et al. Autocatalytic Cleavage of ADAMTS-4 (Aggrecanase-1) Reveals Multiple Glycosaminoglycan-binding Sites J. Biol. Chem., November 1, 2002; 277(45): 42775 - 42780. [Abstract] [Full Text] [PDF] J. E. Sadler A new name in thrombosis, ADAMTS13 PNAS, September 3, 2002; 99(18): 11552 - 11554. [Full Text] [PDF] K. Kokame, M. Matsumoto, K. Soejima, H. Yagi, H. Ishizashi, M. Funato, H. Tamai, M. Konno, K. Kamide, Y. Kawano, et al. Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity PNAS, September 3, 2002; 99(18): 11902 - 11907. [Abstract] [Full Text] [PDF] J. L. Moake Thrombotic Microangiopathies N. Engl. J. Med., August 22, 2002; 347(8): 589 - 600. [Full Text] [PDF] G. Remuzzi, M. Galbusera, M. Noris, M. T. Canciani, E. Daina, E. Bresin, S. Contaretti, J. Caprioli, S. Gamba, P. Ruggenenti, et al. von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome Blood, July 18, 2002; 100(3): 778 - 785. [Abstract] [Full Text] [PDF] V. Bianchi, R. Robles, L. Alberio, M. Furlan, and B. Lammle Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura Blood, June 28, 2002; 100(2): 710 - 713. [Abstract] [Full Text] [PDF] M. Arya, B. Anvari, G. M. Romo, M. A. Cruz, J.-F. Dong, L. V. McIntire, J. L. Moake, and J. A. Lopez Ultralarge multimers of von Willebrand factor form spontaneous high-strength bonds with the platelet glycoprotein Ib-IX complex: studies using optical tweezers Blood, May 13, 2002; 99(11): 3971 - 3977. [Abstract] [Full Text] [PDF] J. N. George, J. E. Sadler, and B. Lammle Platelets: Thrombotic Thrombocytopenic Purpura Hematology, January 1, 2002; 2002(1): 315 - 334. [Abstract] [Full Text] K. Fujikawa, H. Suzuki, B. McMullen, and D. Chung Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family Blood, September 15, 2001; 98(6): 1662 - 1666. [Abstract] [Full Text] [PDF] X. Zheng, D. Chung, T. K. Takayama, E. M. Majerus, J. E. Sadler, and K. Fujikawa Structure of von Willebrand Factor-cleaving Protease (ADAMTS13), a Metalloprotease Involved in Thrombotic Thrombocytopenic Purpura J. Biol. Chem., October 26, 2001; 276(44): 41059 - 41063. [Abstract] [Full Text] [PDF]

	Copyright © 2001 by American Society of Hematology         Online ISSN: 1528-0020