Protein Z-related protease inhibitor

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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.

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

Role in disease

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

History

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

References

^ 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.
^ 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.

v • d • e Proteins: coagulation
Coagulation factors	intrinsic pathway (FXII, FXI, FIX, FVIII) - extrinsic pathway (Tissue factor, FVII) - common pathway (FX, FV, (Pro)thrombin / FII, Fibrin / FI, FXIII) - HMWK - vWF - Kallikrein
Inhibitors	Antithrombin - Protein C - Protein S - Protein Z - ZPI - TFPI
Fibrinolysis	Plasmin - tPA/urokinase - PAI-1/2 - α2-AP - α2-macroglobulin - TAFI

v • d • e Serpins
inhibitory	Alpha 1-antichymotrypsin - Alpha 1-antitrypsin - Alpha 2-antiplasmin - Antithrombin - C1-inhibitor - Heparin cofactor II - Protein C inhibitor - Plasminogen activator inhibitor-1 - Plasminogen activator inhibitor-2 - Protein Z-related protease inhibitor
noninhibitory	Maspin - Ovalbumin - Heat shock protein 47 - Thyroxine-binding globulin - Transcortin