The invention relates to von Willebrand factor (vWF) cleaving protease polypeptide, a nucleic acid molecule encoding the amino acid sequence of a vWF-cp polypeptide and a composition comprising the polypeptide. The invention also relates to the use of the vWF cleaving protease polypeptide for production of vWF cleaving protease polypeptide binding molecules and for production of a preparation for prophylaxis and therapy of thrombosis and thromboembolic disease.The invention relates to von Willebrand factor (vWF) cleaving protease polypeptide, a nucleic acid molecule encoding the amino acid sequence of a vWF-cp polypeptide and a composition comprising the polypeptide. The invention also relates to the use of the vWF cleaving protease polypeptide for production of vWF cleaving protease polypeptide binding molecules and for production of a preparation for prophylaxis and therapy of thrombosis and thromboembolic disease.