Protein Z-related protease inhibitor

File: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.

## Contents

* 1 Role in disease
* 2 History
* 3 References
* 4 External links

## Role in disease

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

## History

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

## References

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1. ↑ 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.
2. ↑ 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.
3. ↑ Han X, Fiehler R, Broze GJ (November 2000). "Characterization of the protein Z-dependent protease inhibitor". Blood. 96 (9): 3049–55. PMID 11049983.

## External links

[edit | edit source]

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

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Proteins involved in coagulation

Coagulation factors|

| Primary hemostasis|

* vWF
* platelet membrane glycoproteins: Ib (A
* B
* IX)
* IIb/IIIa (IIb
* IIIa)
* VI

|

Intrinsic pathway|

* HMWK/Bradykinin
* Prekallikrein/Kallikrein
* XII "Hageman"
* XI
* IX
* VIII

Extrinsic pathway|

* III "Tissue factor"
* VII

Common pathway|

* X
* V
* II "(Pro)thrombin"
* I "Fibrin"
* Fibrinogen (FGA, FGG)
* XIII

Coagulation inhibitors|

* Antithrombin (inhibits II, IX, X, XI, XII)
* Protein C (inhibits V, VIII)/Protein S (cofactor for protein C)
* Protein Z (inhibits X)
* ZPI (inhibits X, XI)
* TFPI (inhibits III)

Thrombolysis/fibrinolysis|

* Plasmin
* tPA/urokinase
* PAI-1/2
* α2-AP
* α2-macroglobulin
* TAFI

* 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
* 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
* SERPINF1
* Transcortin
* Thyroxine-binding globulin
* Angiotensinogen
* SERPINF1

see also disorders of globin and globulin proteins
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