Protein Z-dependent protease inhibitor is a protein circulating in the blood which inhibits factors Xa and XIa of the coagulation cascade. It is a member of the class of the serine protease inhibitors (serpins). Its name implies that it requires protein Z, another circulating protein, to function properly, but this only applies to its inhibition of factor X.
It is about 72 kDa heavy and 444 amino acids large. It is produced by the liver.
Genetics
The gene for ZPI is probably located on the fourteenth chromosome.
Role in disease
Water et al found deficiency of ZPI in 4.4% of a cohort of patients with thrombophilia (a tendency to thrombosis).
History
Han et al first described ZPI in 1998. The same group further characterised it in 2000.
References
- Han X, Fiehler R, Broze GJ. Isolation of protein Z-dependent plasma protease inhibitor. Proc Natl Acad Sci U S A 1998;95:9250-5. Fulltext. PMID 9689066.
- Han X, Fiehler R, Broze GJ. Characterization of the protein Z-dependent protease inhibitor. Blood 2000;96:3049-55. Fulltext. PMID 11049983.
- Water N, Tan T, Ashton F, O'grady A, Day T, Browett P, Ockelford P, Harper P. Mutations within the protein Z-dependent protease inhibitor gene are associated with venous thromboembolic disease: a new form of thrombophilia. Br J Haematol 2004;127:190-4. PMID 15461625.
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