Protein Z-related protease inhibitor

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3F1S.png
Crystal structure of protein Z-dependent peptidase inhibitor (red) in complex with protein Z (blue). Rendered from PDB 3F1S
Identifiers
Symbol SERPINA10
Entrez 51156
HUGO 15996
OMIM 605271
RefSeq NM_016186
UniProt Q9UK55
Other data
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.

It is about 72 kDa heavy and 444 amino acids large. It is produced by the liver.

Role in disease[edit]

Water et al. found deficiency of ZPI in 4.4% of a cohort of patients with thrombophilia (a tendency to thrombosis).[1]

History[edit]

Han et al. first described ZPI in 1998.[2] The same group further characterised it in 2000.[3]

References[edit]

  1. ^ 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.
  2. ^ 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.
  3. ^ Han X, Fiehler R, Broze GJ. Characterization of the protein Z-dependent protease inhibitor. Blood 2000;96:3049-55. Fulltext. PMID 11049983.

External links[edit]

  • The MEROPS online database for peptidases and their inhibitors: I04.005