Protein Z-related protease inhibitor

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.
Crystal structure of protein Z-
dependent peptidase inhibitor (red)
in complex with protein Z (blue).
Contents Rendered from PDB 3F1S (http://w
ww.rcsb.org/pdb/explore/explore.d
Role in disease
o?structureId=3F1S)
History
Identifiers
References
Symbol SERPINA10
External links
NCBI 51156 (https://www.ncbi.nl
gene m.nih.gov/gene?cmd=retri
Role in disease eve&dopt=default&list_uid
s=51156&rn=1)
Water et al. found deficiency of ZPI in 4.4% of a cohort of patients HGNC 15996 (https://www.genen
with thrombophilia (a tendency to thrombosis).[1] ames.org/data/gene-symb
ol-report/#!/hgnc_id/HGN
History C:15996)
OMIM 605271 (https://omim.org/
Han et al. first described ZPI in 1998.[2] The same group further 605271)
characterised it in 2000.[3] RefSeq NM_016186 (https://geno
me.ucsc.edu/cgi-bin/hgTr
References acks?Submit=Submit&pos
ition=NM_016186&rn=1)
1. Van de Water N, Tan T, Ashton F, O'Grady A, Day T,
Browett P, Ockelford P, Harper P (October 2004). UniProt Q9UK55 (https://www.unip
"Mutations within the protein Z-dependent protease rot.org/uniprot/Q9UK55)
inhibitor gene are associated with venous thromboembolic
Other data
disease: a new form of thrombophilia". British Journal of
Haematology. 127 (2): 190–4. doi:10.1111/j.1365- Locus Chr. 14 q32.1 (https://omi
2141.2004.05189.x (https://doi.org/10.1111%2Fj.1365-214 m.org/search/?index=gen
1.2004.05189.x). PMID 15461625 (https://pubmed.ncbi.nl eMap&search=14q32.1)
m.nih.gov/15461625).
 2. Han X, Fiehler R, Broze GJ (August 1998). "Isolation of a
protein Z-dependent plasma protease inhibitor" (https://ww
w.ncbi.nlm.nih.gov/pmc/articles/PMC21324). Proceedings
of the National Academy of Sciences of the United States
of America. 95 (16): 9250–5. doi:10.1073/pnas.95.16.9250
(https://doi.org/10.1073%2Fpnas.95.16.9250). PMC 21324
(https://www.ncbi.nlm.nih.gov/pmc/articles/PMC21324).
PMID 9689066 (https://pubmed.ncbi.nlm.nih.gov/9689066).
3. 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 (https://doi.org/10.1182%2F
blood.V96.9.3049). PMID 11049983 (https://pubmed.ncbi.n
lm.nih.gov/11049983).

External links
The MEROPS online database for peptidases and their inhibitors: I04.005 (http://merops.sange
r.ac.uk/cgi-bin/merops.cgi?id=I04.005)

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This page was last edited on 17 May 2020, at 11:39 (UTC).

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