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Updated: Jul 4, 2026

In Vitro Microfluidic Disease Model to Study Whole Blood-Endothelial Interactions and Blood Clot Dynamics in Real-Time
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Targeting pathogenic VWF/ADAMTS13 dysregulation attenuates CTEPH progression.

Zhijian Wu, Huan Dong, Quan Zhang

    Biorxiv : the Preprint Server for Biology
    |July 3, 2026
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    Summary
    This summary is machine-generated.

    Dysregulation of the von Willebrand factor (VWF) and ADAMTS13 axis drives chronic thromboembolic pulmonary hypertension (CTEPH) by promoting thrombosis and vascular remodeling. Targeting this VWF-ADAMTS13 pathway offers a potential therapeutic strategy for CTEPH.

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    Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet

    Published on: November 4, 2015

    Area of Science:

    • Cardiovascular Biology
    • Hematology
    • Pulmonary Hypertension Research

    Background:

    • Chronic thromboembolic pulmonary hypertension (CTEPH) involves persistent thrombosis and vascular remodeling, but underlying molecular mechanisms are unclear.
    • Elevated von Willebrand factor (VWF) and reduced ADAMTS13 are observed in CTEPH patients, suggesting a role for this axis.
    • The direct contribution of VWF/ADAMTS13 dysregulation to CTEPH pathogenesis requires elucidation.

    Purpose of the Study:

    • To investigate the causative role of VWF-ADAMTS13 axis dysregulation in the progression of chronic thromboembolic pulmonary hypertension (CTEPH).
    • To explore the mechanistic links between VWF-ADAMTS13 imbalance, thrombosis, and vascular remodeling in CTEPH.
    • To evaluate the therapeutic potential of modulating the VWF-ADAMTS13 pathway in CTEPH.

    Main Methods:

    • Established rat models of CTEPH to study VWF and ADAMTS13 dynamics.
    • Utilized genetic manipulation (Adamts13 deficiency, Vwf ablation) and recombinant ADAMTS13 treatment.
    • Assessed thrombus formation, endothelial VWF deposition, platelet recruitment, vascular remodeling, and pulmonary hemodynamics.

    Main Results:

    • CTEPH rats exhibited persistent VWF-rich thrombi, increased endothelial VWF, and reduced ADAMTS13 expression/activity.
    • Genetic Adamts13 deficiency exacerbated thrombosis and mortality; Vwf ablation reduced thrombosis, remodeling, and improved hemodynamics.
    • Recombinant ADAMTS13 treatment decreased UL-VWF, suppressed platelet activation, and prevented thrombosis and remodeling in CTEPH rats.

    Conclusions:

    • VWF-ADAMTS13 axis dysregulation is a key driver of pulmonary thrombosis and vascular remodeling in CTEPH.
    • Ultra-large VWF (UL-VWF) accumulation promotes platelet recruitment and release of pro-remodeling mediators.
    • Targeting the VWF-ADAMTS13 pathway, via recombinant ADAMTS13 or VWF inhibition, represents a promising therapeutic strategy for CTEPH.