Von Willebrand Factor Interacts with Surface-Bound C1q and Induces Platelet Rolling.

Kölm, Robert; Schaller Tschan, Monica; Roumenina, Lubka T; Niemiec, Iga; Kremer Hovinga, Johanna Anna; Khanicheh, Elham; Kaufmann, Beat A; Hopfer, Helmut; Trendelenburg, Marten (2016). Von Willebrand Factor Interacts with Surface-Bound C1q and Induces Platelet Rolling. Journal of immunology, 197(9), pp. 3669-3679. American Association of Immunologists 10.4049/jimmunol.1501876

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Premature atherosclerosis and thrombotic complications are major causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). However, the high incidence of these complications cannot be explained by traditional risk factors alone, suggesting direct effects of an activated immune system on hemostasis. The unexpected nucleotide sequence homology between SLE patient-derived autoantibodies against complement C1q (Fab anti-C1q) and von Willebrand factor (VWF) led us to investigate a potential interaction between the complement and hemostatic systems on the level of initiating molecules. VWF was found to bind to surface-bound C1q under static conditions. The binding could specifically be inhibited by Fab anti-C1q and C1q-derived peptides. Under shear stress the C1q-VWF interaction was enhanced, resembling the binding of VWF to collagen I. Additionally, we could show that C1q-VWF complexes induced platelet rolling and firm adhesion. Furthermore, we observed VWF binding to C1q-positive apoptotic microparticles and cholesterol crystals, as well as increased VWF deposition in C1q-positive glomeruli of SLE patients compared with control nephropathy. We show, to our knowledge for the first time, binding of VWF to C1q and thus a direct interaction between starter molecules of hemostasis and the classical pathway of complement. This direct interaction might contribute to the pathogenic mechanisms in complement-mediated, inflammatory diseases.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Unit Childrens Hospital > Forschungsgruppe Hämatologie (Erwachsene)

UniBE Contributor:

Schaller Tschan, Monica and Kremer Hovinga, Johanna Anna


600 Technology > 610 Medicine & health




American Association of Immunologists




Katrin Kölliker-Schütz

Date Deposited:

20 Feb 2017 15:59

Last Modified:

20 Feb 2017 15:59

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