Protein Z-related protease inhibitor

SERPINA10
Identifiers
Aliases	SERPINA10, PZI, ZPI, serpin family A member 10
External IDs	OMIM: 605271; MGI: 2667725; GeneCards: SERPINA10
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	3F1S, 3H5C, 4AFX, 4AJU
Gene location (Human)
Chr.	Chromosome 14 (human)
End	94,293,268 bp
Gene location (Mouse)
Chr.	Chromosome 12 (mouse)
End	103,597,703 bp
RNA expression pattern
	Top expressed in
	right lobe of liver; ; islet of Langerhans; ; right ovary; ; gastric mucosa; ; left ovary; ; ectocervix; ; muscle layer of sigmoid colon; ; canal of the cervix; ; right uterine tube; ; body of stomach;
	Top expressed in
	left lobe of liver; ; islet of Langerhans; ; yolk sac; ; fetal liver hematopoietic progenitor cell; ; human fetus; ; mesenteric lymph nodes; ; sexually immature organism; ; gallbladder; ; abdominal wall; ; ureter;
	More reference expression data
	n/a
Gene ontology
Molecular function	peptidase inhibitor activity; heparin binding; serine-type endopeptidase inhibitor activity;
Cellular component	extracellular region; extracellular exosome; extracellular space; endoplasmic reticulum lumen;
Biological process	hemostasis; bioluminescence; regulation of acrosome reaction; negative regulation of peptidase activity; blood coagulation; negative regulation of endopeptidase activity; post-translational protein modification;
	Sources:Amigo / QuickGO
Orthologs
Databases	NCBI: entry; OMA: entry
	51156
	217847
	ENSG00000140093; ENSG00000278767
	ENSMUSG00000061947
	Q9UK55
	Q8R121
	NM_001100607; NM_016186
	NM_001301404; NM_144834
	NP_001094077; NP_057270
	NP_001288333; NP_659083
	Wikidata
View/Edit Human	View/Edit Mouse

Protein Z-dependent protease inhibitor (ZPI) is a protein that in humans is encoded by the gene SERPINA10. ZPI circulates in the blood and 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).

History

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

References

1 2 3 ENSG00000278767 GRCh38: Ensembl release 89: ENSG00000140093, ENSG00000278767 – Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000061947 – Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ 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

[refGRCh38Ensembl-1] 1 2 3 ENSG00000278767 GRCh38: Ensembl release 89: ENSG00000140093, ENSG00000278767 – Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000061947 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid15461625-5] 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.

[pmid9689066-6] 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.

[pmid11049983-7] 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.

v t e Serpins
inhibitory	Alpha 1-antichymotrypsin Alpha-1 antitrypsin Alpha 2-antiplasmin Antithrombin C1-inhibitor CU-2010 and CU-2020 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