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Showing 1 to 4 of 4 for “"ADAMTS13-VWF"”.

  1. A genome-wide association study in chronic thromboembolic pulmonary hypertension and the ADAMTS13-VWF axis

    … variation in plasma von Willebrand factor (VWF) levels. Abnormalities in haemostasis are implicated in CTEPH pathobiology, including elevated levels of VWF, which is cleaved by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). The ADAMTS13-VWF axis …

    cambridge Repository record for A genome-wide association study in chronic thromboembolic pulmonary hypertension and the ADAMTS13-VWF axis (opens in a new tab)

  2. PATHOPHYSIOLOGY OF VON WILLEBRAND FACTOR IN BLEEDING AND THROMBOSIS

    von Willebrand factor (VWF) is a multimeric glycoprotein mainly known to be involved in primary hemostasis recruiting platelets at the site of damaged vessels and acting as factor VIII (FVIII) carrier. Quantitative or qualitative alteration of VWF protein is responsible for von Willebrand disease …

    milano Repository record for PATHOPHYSIOLOGY OF VON WILLEBRAND FACTOR IN BLEEDING AND THROMBOSIS (opens in a new tab)

  3. The effect of single nucleotide polymorphisms and mutations on congenital thrombotic thrombocytopenic purpura phenotype

    … of the von Willebrand factor cleaving protease (ADAMTS13). Over 100 mutations have been identified in ADAMTS13 yet, in some cases, these mutations in congenital TTP alone do not explain the disease phenotype, particularly in late-onset congenital TTP. One aim of this study was to analyse the …

    westminster Repository record for The effect of single nucleotide polymorphisms and mutations on congenital thrombotic thrombocytopenic purpura phenotype (opens in a new tab)

  4. Unraveling the mechanism of ADAMTS13 resistance to protease inhibition

    Background: ADAMTS13 is a metalloprotease that regulates the delicate balance between VWF multimeric length and its platelet capturing capacity. Unlike other ADAMTS and coagulation proteases, ADAMTS13 exhibits a prolonged half-life of several days as an active protease, suggesting that it is …

    mcmaster Repository record for Unraveling the mechanism of ADAMTS13 resistance to protease inhibition (opens in a new tab)