Von Willebrand Factor (VWF): Biochemical and molecular characteristics

The VWF is an adhesive glycoprotein present in different cell types such as platelets, endothelial cells, megacariocites, and so on. It is synthesized in a precursor molecule form that, once splited, releases an amino acid propeptide of 741 aa leading to a large mature subunit (2,050 amino acids). Through a disulfide bond, this subunit is capable of forming multimeric structures with variable sizes reaching the 100 monomers and a molecular weight of the order of 10,000 kilodaltons (Figure 1).

The transcript of the VWF gene generates about 9 kilobases of mRNA that encodes a precursor of 2,813 amino acids known as pre-pro-VWF. This mRNA is transcribed from a single gene located on the telomeric end of the short arm of chromosome 12 (position 12p12). The VWF gene extends about 178 kb in the genome and contains a total of 52 exons, with measures ranging between 40 and 1,400 base pairs (bp), making it one of the largest and most complex genes described in human (Figure 1) [1, 2].