作者: Montgomery RR Flood VH , Schlauderaff AC , Haberichter SL , Slobodianuk TL , Jacobi PM
DOI: 10.1182/BLOOD-2014-11-610824
关键词:
摘要: Von Willebrand factor (VWF) contains binding sites for platelets and vascular collagens to facilitate clot formation at of injury. Although previous work has shown that VWF can bind type IV collagen (collagen 4), little characterization this interaction been performed. We examined the 4 in vitro extended a murine model defective VWF–collagen interactions. The interactions were further studied using plasma samples from large study both healthy controls subjects with different types von disease (VWD). Our results show appears exclusively via A1 domain, specific sequence variations identified through patient site-directed mutagenesis domain decrease or abrogate interaction. In addition, VWF-dependent platelet under flow conditions requires an intact domain. observed decreased was associated select 1 2M VWD. This suggests additional mechanism which variants may alter hemostasis.