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Prepublished online as a Blood First Edition Paper on July 12, 2002; DOI 10.1182/blood-2002-05-1397. This Article Full Text (PDF) All Versions of this Article: 2002-05-1397v1 100/10/3626    most recent Alert me when this article is cited Alert me if a correction is posted 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 Plaimauer, B. Articles by Scheiflinger, F. Search for Related Content PubMed PubMed Citation Articles by Plaimauer, B. Articles by Scheiflinger, F. Social Bookmarking                   What's this? Submitted May 15, 2002 Accepted July 2, 2002 Cloning, Expression and Functional Characterization of the von Willebrand Factor-Cleaving Protease (ADAMTS13) Barbara Plaimauer, Klaus Zimmermann, Dirk Voelkel, Gerhard Antoine, Randolf Kerschbaumer, Pegah Jenab, Miha Furlan, Helen Gerritsen, Bernhard Laemmle, Hans Peter Schwarz, and Friedrich Scheiflinger* Biomedical Research Center, Baxter BioScience, Orth/Donau, Austria Baxter BioScience, Vienna, Austria Central Hematology Laboratory, Inselspital Bern, Bern, Switzerland * Corresponding author; email: scheiff{at}baxter.com. Deficient von Willebrand factor (vWF) degradation has been associated with thrombotic thrombocytopenic purpura (TTP). In hereditary TTP the specific vWF-cleaving protease (vWF-cp) is absent or functionally defective, whereas in the non-familial, acquired form of TTP an autoantibody inhibiting the vWF-cp activity is found transiently in most patients. The 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. To establish the functional activity of the recombinant vWF-cp, we cloned the complete cDNA sequence in an eukaryotic expression vector and transiently expressed the encoded recombinant ADAMTS13 in HEK 293 cells. The expressed protein degraded vWF multimers and proteolytically cleaved vWF to the same fragments as those generated by plasma vWF-cp. Furthermore, recombinant ADAMTS13-mediated degradation of vWF multimers was entirely inhibited in the presence of a plasma from a patient with acquired TTP. These data show that ADAMTS13 is responsible for the physiologic proteolytic degradation of vWF multimers. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: F. Yu, Y. Tan, and M.-H. 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