Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

Vitamin K-dependent protein Z

The PROZ gene encodes protein Z, a regulator of the coagulation system which together with cofactor encoded by the SERPINA10 gene inhibits activated coagulations factors Xa and XIa. Inactivating mutations may promote thrombophilia.

Genetests:

Clinic Method Carrier testing
Turnaround 5
Specimen type genomic DNA
Research Method Genomic sequencing of the entire coding region
Turnaround 25
Specimen type genomic DNA
Clinic Method Massive parallel sequencing
Turnaround 25
Specimen type genomic DNA

Related Diseases:

Protein Z deficiency
PROZ
SERPINA10

References:

1.

Prowse CV et. al. (1977) The isolation of a new warfarin-sensitive protein from bovine plasma.

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2.

Hogg PJ et. al. (1991) Interaction of human protein Z with thrombin: evaluation of the species difference in the interaction between bovine and human protein Z and thrombin.

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3.

Ichinose A et. al. (1990) Amino acid sequence of human protein Z, a vitamin K-dependent plasma glycoprotein.

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4.

Højrup P et. al. (1985) Amino acid sequence of bovine protein Z: a vitamin K-dependent serine protease homolog.

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5.

Broze GJ et. al. (1984) Human Protein Z.

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6.

Højrup P et. al. (1982) Amino-acid sequence of the vitamin-K-dependent part of protein Z.

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7.

Fujimaki K et. al. (1998) The gene for human protein Z is localized to chromosome 13 at band q34 and is coded by eight regular exons and one alternative exon.

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8.

Han X et. al. (1998) Isolation of a protein Z-dependent plasma protease inhibitor.

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9.

Yin ZF et. al. (2000) Prothrombotic phenotype of protein Z deficiency.

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10.

Vasse M et. al. (2001) Frequency of protein Z deficiency in patients with ischaemic stroke.

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11.

None (2001) Protein Z-dependent regulation of coagulation.

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12.

Lichy C et. al. (2004) A common polymorphism of the protein Z gene is associated with protein Z plasma levels and with risk of cerebral ischemia in the young.

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13.

Souri M et. al. (2005) A naturally occurring E30Q mutation in the Gla domain of protein Z causes its impaired secretion and subsequent deficiency.

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14.

Kemkes-Matthes B et. al. (2005) R255h amino acid substitution of protein Z identified in patients with factor V Leiden mutation.

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15.

Santacroce R et. al. (2006) Low protein Z levels and risk of occurrence of deep vein thrombosis.

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Update: Sept. 26, 2018