Domain structure of human and hagfish VWF. (A) Schematic representation of the overall domain structure of human and hagfish VWF based on recently revised domain assignments.28 Shown are annotations of the furin cleavage site (solid arrow), the heparin, FVIII, GPIbα, and collagen binding sites, the RGD recognition site for GPIIb/IIIa, the ADAMTS13 cleavage site (dashed arrow), and known human exons. Note that hagfish Vwf lacks the A3 domain and the large adjacent multidomain region, D4N-VWD4-C8-4-TIL-4-C1.C, C-terminal end; N, N-terminal end. (B) Amino acid alignments of VWF sequences from varied vertebrate species at the furin cleavage site (red arrowhead). (C) Amino acid alignments of VWF sequences from varied vertebrate species at the ADAMTS13 cleavage site (red arrowhead). ADAMTS13, a disintegrin and metalloproteinase with thrombospondin motifs 13; RGD, Arg-Gly-Asp.
