Protein Z-related protease inhibitor

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.

Crystal structure of protein Z-dependent peptidase inhibitor (red) in complex with protein Z (blue). Rendered from PDB 3F1S
Identifiers
Symbol	SERPINA10
NCBI gene	51156
HGNC	15996
OMIM	605271
RefSeq	NM_016186
UniProt	Q9UK55
Other data
Locus	Chr. 14 q32.1
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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]