Protein Z-related protease inhibitor
Crystal structure of protein Z-dependent peptidase inhibitor (red) in complex with protein Z (blue). Rendered from PDB 3F1S
|Locus||Chr. 14 q32.1|
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.
Role in disease
- Van de Water N, Tan T, Ashton F, O'Grady A, Day T, Browett P, Ockelford P, Harper P (October 2004). "Mutations within the protein Z-dependent protease inhibitor gene are associated with venous thromboembolic disease: a new form of thrombophilia". British Journal of Haematology. 127 (2): 190–4. doi:10.1111/j.1365-2141.2004.05189.x. PMID 15461625.
- Han X, Fiehler R, Broze GJ (August 1998). "Isolation of a protein Z-dependent plasma protease inhibitor". Proceedings of the National Academy of Sciences of the United States of America. 95 (16): 9250–5. PMC 21324. PMID 9689066.
- Han X, Fiehler R, Broze GJ (November 2000). "Characterization of the protein Z-dependent protease inhibitor". Blood. 96 (9): 3049–55. PMID 11049983.