Protein Z-related protease inhibitor
- Protein Z-related protease inhibitor
protein
Name =
caption =
width =
HGNCid = 15996
Symbol = SERPINA10
AltSymbols =
EntrezGene = 51156
OMIM = 605271
RefSeq = NM_016186
UniProt = Q9UK55
PDB =
ECnumber =
Chromosome = 14
Arm = q
Band = 32.1
LocusSupplementaryData = 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.
Role in disease
Water "et al" found deficiency of ZPI in 4.4% of a cohort of patients with thrombophilia (a tendency to thrombosis). [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.]
History
Han "et al" first described ZPI in 1998. [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. [http://www.pnas.org/cgi/content/full/95/16/9250 Fulltext] . PMID 9689066.] The same group further characterised it in 2000. [Han X, Fiehler R, Broze GJ. "Characterization of the protein Z-dependent protease inhibitor". Blood 2000;96:3049-55. [http://www.bloodjournal.org/cgi/content/full/96/9/3049 Fulltext] . PMID 11049983.]
References
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2010.
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