Biology and physics of von Willebrand factor concatamers.

  title={Biology and physics of von Willebrand factor concatamers.},
  author={Timothy A Springer},
  journal={Journal of thrombosis and haemostasis : JTH},
  volume={9 Suppl 1},
Structural specialisations enable von Willebrand factor (VWF) to assemble during biosynthesis into helical tubules in Weibel-Palade bodies (WPB). Specialisations include a pH-regulated dimeric bouquet formed by the C-terminal half of VWF and helical assembly guided by the N-terminal half that templates inter-dimer disulphide bridges. Orderly assembly and storage of ultra-long concatamers in helical tubules, without crosslinking of neighboring tubules, enables unfurling during secretion without… CONTINUE READING