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Prepublished online as a Blood First Edition Paper on July 12, 2002; DOI 10.1182/blood-2002-05-1397.
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Blood, 15 November 2002, Vol. 100, No. 10, pp. 3626-3632
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Cloning, expression, and functional characterization of the von
Willebrand factor-cleaving protease (ADAMTS13)
Barbara Plaimauer,
Klaus Zimmermann,
Dirk Völkel,
Gerhard Antoine,
Randolf Kerschbaumer,
Pegah Jenab,
Miha Furlan,
Helen Gerritsen,
Bernhard Lämmle,
Hans Peter Schwarz, and
Friedrich Scheiflinger
From Baxter BioScience, Biomedical Research Center,
Orth, Austria; and Central Hematology Laboratory, University Hospital,
Inselspital, Bern, Switzerland.
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 nonfamilial, acquired form of
TTP, an autoantibody inhibiting 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 recombinant VWF-cp, we cloned the complete cDNA sequence in
a 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 plasma from a patient with acquired TTP. These data
show that ADAMTS13 is responsible for the physiologic proteolytic degradation of VWF multimers.
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