A disintegrin and metalloproteinase with a thrombospondin type i motif, member 13 (adamts-13) mutants, compositions and therapeutic methods thereof

A mutant and/or variant of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS-13) that carries at least one mutation, or any truncated variant thereof, wherein said mutant displays resistance and/or reduced sensitivity to cleavage and/or inactivation by at least one tissue plasminogen activator (tPA), or any mutant or variant thereof.

A method for the treatment, amelioration, inhibition or prophylaxis of a disease, disorder, or condition associated with coagulation in a subject in need thereof, the method comprising the step of administering to said subject a therapeutically effective amount of at least one ADAMTS-13 mutant and/or variant, or any truncated variant thereof, or any composition or combined composition comprising said mutant, wherein said mutant carries at least one mutation and displays resistance and/or reduced sensitivity to cleavage and/or inactivation, by at least one tPA, or any mutant or variant thereof.

A kit comprising:

• (a) at least one ADAMTS-13 mutant and/or variant and/or any truncated variant thereof, or any composition thereof, optionally, in a first dosage form, wherein said mutant or any truncated variant thereof, carries at least one mutation and displays resistance and/or reduced sensitivity to cleavage by at least one tPA, or any mutant or variant thereof;
• and
• (b) at least one tPA or any functional fragments or variants thereof, or any composition thereof, optionally, in a second dosage form.