von Willebrand factor is a cofactor in complement regulation
| Autor: | Xiaowen Liang, Dominic W. Chung, Shuju Feng, Vahid Afshar-Kharghan, Michael H. Kroll |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Immunology
Cleavage (embryo) Biochemistry Complement factor B Von Willebrand factor hemic and lymphatic diseases Complement Factor D Atypical hemolytic uremic syndrome von Willebrand Factor medicine Humans Complement Activation biology Chemistry Fibrinogen Cell Biology Hematology Complement System Proteins medicine.disease Complement system Cell biology Complement (complexity) biology.protein iC3b Protein Multimerization circulatory and respiratory physiology |
| Zdroj: | Blood. 125(6) |
| ISSN: | 1528-0020 |
| Popis: | Several complement proteins interact with hemostatic factors. We discovered that von Willebrand factor (VWF) acts as a cofactor for factor I–mediated cleavage of complement C3b, thereby shutting down complement activation. The complement regulatory function of VWF multimers depends on their size. Smaller VWF multimers enhance cleavage of C3b but large and ultra-large VWF (ULVWF) multimers have no effect on C3b cleavage and permit default complement activation. We conclude that normal plasma VWF multimers prevent complement activation and steer the complement pathway toward generation of inactivated C3b (iC3b). ULVWF multimers, as are present in patients with thrombotic microangiopathy, lack an inhibitory effect on complement and permit complement activation. |
| Databáze: | OpenAIRE |
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