Fig. 5.
Consensus sequence for the cleavage site of propeptide from mature VWF by the processing enzyme PACE-furin, characterized by a functionally important Lys (K) at position −2 and Arg (R) at position −4 upstream of the cleavage site (R-S).
The Cys2527Thr mutation falls at position −4 and changes the Arg to a Cys.