Protein Z-related protease inhibitor

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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.

Quick facts Identifiers, Symbol ...
Crystal structure of protein Z-dependent peptidase inhibitor (red) in complex with protein Z (blue). Rendered from PDB 3F1S
Identifiers
SymbolSERPINA10
NCBI gene51156
HGNC15996
OMIM605271
RefSeqNM_016186
UniProtQ9UK55
Other data
LocusChr. 14 q32.1
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StructuresSwiss-model
DomainsInterPro
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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

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