Cleavage of von Willebrand factor by ADAMTS-13 in vitro: effect of temperature and barium ions on the proteolysis kinetics

Blood Coagul Fibrinolysis. 2005 Nov;16(8):607-11. doi: 10.1097/01.mbc.0000187251.32630.1f.

Abstract

The multimeric size of von Willebrand factor (VWF) is regulated by the specific cleaving metalloprotease, ADAMTS-13. Laboratory assays for ADAMTS-13 are useful for identifying severe protease-deficient activity. ADAMTS-13 activity is currently assayed by prolonged dialysis of plasma at 37 degrees C in low-ionic-strength denaturing buffer. We investigated the effect of temperature and divalent cation supplementation on the kinetics of VWF proteolysis by ADAMTS-13 in vitro. Proteolysis was monitored for 24 h at 37, 22, and 4 degrees C, in the presence or absence of barium ions, by measuring the binding of VWF to collagen. Complete VWF proteolysis was observed at 37 degrees C in the presence of BaCl2, while about 25% VWF still bound to collagen when BaCl2 supplementation was omitted. Proteolysis kinetics at 22 and 4 degrees C was slower but complete, even in the absence of added barium. A subphysiological temperature might influence the proteolysis kinetics by determining minor variations of the ADAMTS-13 structure, or further modification of the VWF substrate. We describe a simple procedure to analyse the kinetics of VWF proteolysis that is suitable for routine diagnostic use. Furthermore, we offer new insight into the biochemistry of ADAMTS-13.

MeSH terms

  • ADAM Proteins / chemistry*
  • ADAM Proteins / deficiency
  • ADAMTS13 Protein
  • Barium Compounds / chemistry*
  • Chlorides / chemistry*
  • Hot Temperature
  • Humans
  • Ions / chemistry
  • Kinetics
  • von Willebrand Factor / chemistry*

Substances

  • Barium Compounds
  • Chlorides
  • Ions
  • von Willebrand Factor
  • barium chloride
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human