Protein Z-related protease inhibitor

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Crystal structure of protein Z-dependent peptidase inhibitor (red) in complex with protein Z (blue). Rendered from PDB 3F1S

Identifiers

Symbol

SERPINA10

Other data

Chr. 14 q32.1

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Structures	Swiss-model
Domains	InterPro

Protein Z-dependent protease inhibitor (ZPI) 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

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

^ 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. Bibcode:1998PNAS...95.9250H. doi:10.1073/pnas.95.16.9250. 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. doi:10.1182/blood.V96.9.3049. PMID 11049983.

External links

The MEROPS online database for peptidases and their inhibitors: I04.005 Archived 2020-05-31 at the Wayback Machine

Coagulation cascade

Coagulation factors

Primary hemostasis (platelet activation)	von Willebrand factor (vWF) Platelet membrane glycoproteins: Glycoprotein Ib (GPIb) (GP1BA GP1BB Glycoprotein IX (GP9)) Glycoprotein IIb/IIIa (GPIIb GPIIIa) Glycoprotein VI (GPVI)
Intrinsic pathway (contact activation)	High-molecular-weight kininogen (HMWK) Bradykinin Prekallikrein Kallikrein Factor XII (Hageman factor) Factor XI Factor IX Factor VIII
Extrinsic pathway (tissue factor)	Factor III (tissue factor) Factor VII
Common pathway	Factor X Factor V Prothrombin (factor II) Thrombin (factor IIa) Fibrinogen (factor I) (FGA, FGG) Fibrin (factor Ia) Factor XIII

Anticoagulant factors

Fibrinolytic factors

Coagulation markers

Platelet activation	Platelet factor 4 (PF4) β-Thromboglobulin (β-TG)
Thrombin generation	Prothrombin fragment 1+2 (F1+2) Thrombin–antithrombin complex (TAT) Activated protein C–protein C inhibitor (APC–PCI)
Fibrin generation	Fibrinopeptide A (FpA) Fibrinopeptide B (FpB) Fibrin monomers (FM)
Fibrinolysis	Plasmin-α₂-antiplasmin complex (PAP) D-Dimer (DD)

v t 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 SERPINA1 SERPINA2 SERPINA3 SERPINA4 SERPINA5 SERPINA9 SERPINA14 SERPINB1 SERPINB2 SERPINB3 SERPINB4 SERPINB5 SERPINB6 SERPINB7 SERPINB8 SERPINB9 SERPINB10 SERPINB13 SERPINC1 SERPIND1 SERPINE1 SERPINE2 SERPINE2 SERPINF1 SERPING1 SERPINH1 SERPINI1 SERPINI2
Cross class inhibitory	MENT SCCA-1 CrmA
noninhibitory	Heat shock protein 47 Maspin Ovalbumin Transcortin Thyroxine-binding globulin Angiotensinogen PEDF
see also disorders of globin and globulin proteins

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