SERPINA10 serpin family A member 10 [Homo sapiens (human)] - Gene

Summary

Official Symbol: SERPINA10provided by HGNC
Official Full Name: serpin family A member 10provided by HGNC
Primary source: HGNC:HGNC:15996
See related: Ensembl:ENSG00000140093 MIM:605271; AllianceGenome:HGNC:15996
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Homo sapiens
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as: PZI; ZPI
Summary: The protein encoded by this gene belongs to the serpin family. It is predominantly expressed in the liver and secreted in plasma. It inhibits the activity of coagulation factors Xa and XIa in the presence of protein Z, calcium and phospholipid. Mutations in this gene are associated with venous thrombosis. Alternatively spliced transcript variants have been found for this gene.[provided by RefSeq, May 2010]
Expression: Restricted expression toward liver (RPKM 44.6) See more
Orthologs: mouse all
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Genomic context

Location:: 14q32.13

Exon count:: 6

Annotation release	Status	Assembly	Chr	Location
RS_2024_08	current	GRCh38.p14 (GCF_000001405.40)	14	NC_000014.9 (94280460..94293268, complement)
RS_2024_08	current	T2T-CHM13v2.0 (GCF_009914755.1)	14	NC_060938.1 (88507743..88520553, complement)
RS_2024_09	previous assembly	GRCh37.p13 (GCF_000001405.25)	14	NC_000014.8 (94746797..94759605, complement)

Chromosome 14 - NC_000014.9 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

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Expression

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See details

Project title: Tissue-specific circular RNA induction during human fetal development
Description: 35 human fetal samples from 6 tissues (3 - 7 replicates per tissue) collected between 10 and 20 weeks gestational time were sequenced using Illumina TruSeq Stranded Total RNA
BioProject: PRJNA270632
Publication: PMID 26076956
Analysis date: Mon Apr 2 22:54:59 2018

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Heparin activation of protein Z-dependent protease inhibitor (ZPI) allosterically blocks protein Z activation through an extended heparin-binding site.
Title: Heparin activation of protein Z-dependent protease inhibitor (ZPI) allosterically blocks protein Z activation through an extended heparin-binding site.
Plasma concentration of protein Z-dependent protease inhibitor and ZPI exon 3 mutations in preeclampsia.
Title: Plasma concentration of protein Z-dependent protease inhibitor and ZPI exon 3 mutations in preeclampsia.
these results suggest an important anticoagulant role for the ZPI-PZ complex in regulating both free FXa generated in the initiation phase of coagulation as well as prothrombinase-bound FXa in the propagation phase that complement prothrombinase regulation by APC.
Title: Protein Z-dependent protease inhibitor (ZPI) is a physiologically significant inhibitor of prothrombinase function.
PZ/ZPI polymorphisms do not appear to play an active role in the development of prosthesis heart valve thrombosis.
Title: The Role of Protein Z and Protein Z-Dependent Protease Inhibitor Polymorphisms in the Development of Prosthetic Heart Valve Thrombosis.
Data suggest oxidized lipid vesicles with phosphatidylserine/polyunsaturated fatty acids promote inactivation of ZPI-PZ complex or free ZPI; binding of PZ-complexed or free ZPI to oxidized vesicles mediates inactivation of ZPI (an inhibitor of FXa); blocking heparin- (anticoagulant-)binding site on ZPI interferes with binding to lipid or PZ. (ZPI = protein Z-dependent protease inhibitor; PZ = protein Z; FXa = factor Xa)
Title: Lipid oxidation inactivates the anticoagulant function of protein Z-dependent protease inhibitor (ZPI).
rs2232710 SNV showed no association with DVT in two Dutch replication cohorts the LETS study and the MEGA study, indicating that the rs2232710 variant is not a risk factor for DVT
Title: Single Nucleotide Variant rs2232710 in the Protein Z-Dependent Protease Inhibitor (ZPI, SERPINA10) Gene Is Not Associated with Deep Vein Thrombosis.
Results show the energetic basis of the Z-dependent protease inhibitor (ZPI)-protein Z (PZ) interaction and suggest an important role for ZPI Lys-239 in PZ catalytic action.
Title: Thermodynamic and kinetic characterization of the protein Z-dependent protease inhibitor (ZPI)-protein Z interaction reveals an unexpected role for ZPI Lys-239.
The unbalance between PZ and ZPI in plasma samples from patients with Colorectal Cancer or Pancreatic Cancer is not only related to an inflammatory state but could also results from an ectopic synthesis of these proteins by the cancer cells.
Title: Unbalance between plasma levels of Protein Z and protein Z-dependent inhibitor in patients with colorectal and pancreatic cancer: a pilot study.
Protein Z EGF2 subdomain constitutes an interactive-site for ZPI protein.
Title: Characterization of the protein Z-dependent protease inhibitor interactive-sites of protein Z.
Protein Z/protein Z-dependent protease inhibitor and Fxa expression in human gastric cancer cells indicate that these proteins may play a role in anticoagulant events at the tumor tissue.
Title: Protein Z/protein Z-dependent protease inhibitor system in loco in human gastric cancer.

Submit: New GeneRIF Correction See all GeneRIFs (32)

Phenotypes

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Find tests for this gene in the NIH Genetic Testing Registry (GTR)

Review eQTL and phenotype association data in this region using PheGenI

Variation

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See variants in ClinVar

See studies and variants in dbVar

See Variation Viewer (GRCh37.p13)

See Variation Viewer (GRCh38)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Gene Ontology Provided by GOA

Function	Evidence Code	Pubs
enables heparin binding	IEA Inferred from Electronic Annotation more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed

Process	Evidence Code	Pubs
involved_in blood coagulation	IEA Inferred from Electronic Annotation more info
involved_in liver regeneration	IEA Inferred from Electronic Annotation more info
NOT involved_in regulation of acrosome reaction	IDA Inferred from Direct Assay more info	PubMed

Component	Evidence Code	Pubs
located_in endoplasmic reticulum lumen	TAS Traceable Author Statement more info
located_in extracellular exosome	HDA more info	PubMed
is_active_in extracellular space	IBA Inferred from Biological aspect of Ancestor more info

General protein information

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Preferred Names: protein Z-dependent protease inhibitor

Names: PZ-dependent protease inhibitor; Protein-Z dependent proteinase inhibitor; serine (or cysteine) proteinase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 10; serpin A10; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 10

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

NG_021202.2 RefSeqGene

Range

4992..17800

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GenBank, FASTA, Sequence Viewer (Graphics), LRG_576

mRNA and Protein(s)

NM_001100607.3 → NP_001094077.1 protein Z-dependent protease inhibitor precursor

See identical proteins and their annotated locations for NP_001094077.1

Status: REVIEWED

Description

Transcript Variant: This variant (2) differs in the 5' UTR compared to variant 1. Variants 1 and 2 encode the same protein.

Source sequence(s)

AF181467, AL117259, BC022261

Consensus CDS

CCDS9923.1

UniProtKB/Swiss-Prot

A5Z2A5, Q6UWX9, Q86U20, Q9UK55

UniProtKB/TrEMBL

B2RBZ5

Related

ENSP00000261994.4, ENST00000261994.9

Conserved Domains (1) summary

cd02055
Location:74 → 438

PZI; Protein Z-dependent protease inhibitor (ZPI) is a member of the serpin superfamily of proteinase inhibitors (clade A10). ZPI inhibits coagulation factor Xa , dependent on protein Z (PZ), a vitamin K-dependent plasma protein. ZPI also inhibits factor XIa ...
NM_016186.3 → NP_057270.1 protein Z-dependent protease inhibitor precursor

See identical proteins and their annotated locations for NP_057270.1

Status: REVIEWED

Description

Transcript Variant: This variant (1) represents the longer transcript. Variants 1 and 2 encode the same protein.

Source sequence(s)

AF181467, AI174900, AL117259

Consensus CDS

CCDS9923.1

UniProtKB/Swiss-Prot

A5Z2A5, Q6UWX9, Q86U20, Q9UK55

UniProtKB/TrEMBL

B2RBZ5

Related

ENSP00000376809.1, ENST00000393096.5

Conserved Domains (1) summary

cd02055
Location:74 → 438

PZI; Protein Z-dependent protease inhibitor (ZPI) is a member of the serpin superfamily of proteinase inhibitors (clade A10). ZPI inhibits coagulation factor Xa , dependent on protein Z (PZ), a vitamin K-dependent plasma protein. ZPI also inhibits factor XIa ...

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2024_08

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p14 Primary Assembly

Genomic

NC_000014.9 Reference GRCh38.p14 Primary Assembly

Range

94280460..94293268 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_017021353.2 → XP_016876842.1 protein Z-dependent protease inhibitor isoform X1

UniProtKB/TrEMBL

B2RBZ5, G3V2W1

Related

ENSP00000450896.1, ENST00000554723.5

Conserved Domains (1) summary

cd02055
Location:114 → 478

PZI; Protein Z-dependent protease inhibitor (ZPI) is a member of the serpin superfamily of proteinase inhibitors (clade A10). ZPI inhibits coagulation factor Xa , dependent on protein Z (PZ), a vitamin K-dependent plasma protein. ZPI also inhibits factor XIa ...
XM_005267733.6 → XP_005267790.1 protein Z-dependent protease inhibitor isoform X2

See identical proteins and their annotated locations for XP_005267790.1

UniProtKB/Swiss-Prot

A5Z2A5, Q6UWX9, Q86U20, Q9UK55

UniProtKB/TrEMBL

B2RBZ5

Conserved Domains (1) summary

cd02055
Location:74 → 438

PZI; Protein Z-dependent protease inhibitor (ZPI) is a member of the serpin superfamily of proteinase inhibitors (clade A10). ZPI inhibits coagulation factor Xa , dependent on protein Z (PZ), a vitamin K-dependent plasma protein. ZPI also inhibits factor XIa ...

Reference GRCh38.p14 ALT_REF_LOCI_1

Genomic

NT_187601.1 Reference GRCh38.p14 ALT_REF_LOCI_1

Range

1395022..1407830 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_054328978.1 → XP_054184953.1 protein Z-dependent protease inhibitor isoform X1

UniProtKB/TrEMBL

B2RBZ5, G3V2W1
XM_054328979.1 → XP_054184954.1 protein Z-dependent protease inhibitor isoform X2

UniProtKB/Swiss-Prot

A5Z2A5, Q6UWX9, Q86U20, Q9UK55

UniProtKB/TrEMBL

B2RBZ5

Alternate T2T-CHM13v2.0

Genomic

NC_060938.1 Alternate T2T-CHM13v2.0

Range

88507743..88520553 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_054376169.1 → XP_054232144.1 protein Z-dependent protease inhibitor isoform X1

UniProtKB/TrEMBL

B2RBZ5
XM_054376170.1 → XP_054232145.1 protein Z-dependent protease inhibitor isoform X2

UniProtKB/TrEMBL

B2RBZ5