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

Gene ID: 51156, updated on 3-Apr-2024

Summary

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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
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Genomic context

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See SERPINA10 in Genome Data Viewer
Location:
14q32.13
Exon count:
6
Annotation release Status Assembly Chr Location
RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 14 NC_000014.9 (94280460..94293268, complement)
RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 14 NC_060938.1 (88507743..88520553, complement)
105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 14 NC_000014.8 (94746797..94759605, complement)

Chromosome 14 - NC_000014.9Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC105370634 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr14:94639893-94640455 Neighboring gene protein phosphatase 4 regulatory subunit 4 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr14:94757989-94758488 Neighboring gene serpin family A member 6 Neighboring gene BRD4-independent group 4 enhancer GRCh37_chr14:94812301-94813500 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr14:94828015-94828515 Neighboring gene serpin family A member 2 (gene/pseudogene)

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018

Bibliography

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Related articles in PubMed

  1. Heparin activation of protein Z-dependent protease inhibitor (ZPI) allosterically blocks protein Z activation through an extended heparin-binding site. Huang X, et al. J Biol Chem, 2022 Jun. PMID 35551912, Free PMC Article
  2. Plasma concentration of protein Z-dependent protease inhibitor and ZPI exon 3 mutations in preeclampsia. Zhang P, et al. J Obstet Gynaecol, 2021 Oct. PMID 33427552
  3. Protein Z-dependent protease inhibitor (ZPI) is a physiologically significant inhibitor of prothrombinase function. Huang X, et al. J Biol Chem, 2019 May 10. PMID 30918026, Free PMC Article
  4. Single Nucleotide Variant rs2232710 in the Protein Z-Dependent Protease Inhibitor (ZPI, SERPINA10) Gene Is Not Associated with Deep Vein Thrombosis. Gorski MM, et al. PLoS One, 2016. PMID 26982741, Free PMC Article
  5. Characterization of the heparin-binding site of the protein z-dependent protease inhibitor. Yang L, et al. Biochemistry, 2012 May 15. PMID 22540147, Free PMC Article

See all (49) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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).
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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)

Genotypes

Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

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 
enables serine-type endopeptidase inhibitor activity IBA
Inferred from Biological aspect of Ancestor
more info
  
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
  
involved_in negative regulation of endopeptidase activity IBA
Inferred from Biological aspect of Ancestor
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 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

  1. NG_021202.2 RefSeqGene

    Range
    4992..17800
    Download
    GenBank, FASTA, Sequence Viewer (Graphics), LRG_576

mRNA and Protein(s)

  1. 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 ...

  2. 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_2023_10

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

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000014.9 Reference GRCh38.p14 Primary Assembly

    Range
    94280460..94293268 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. 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 ...

  2. 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

  1. NT_187601.1 Reference GRCh38.p14 ALT_REF_LOCI_1

    Range
    1395022..1407830 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

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

    UniProtKB/TrEMBL
    G3V2W1

  2. XM_054328979.1 → XP_054184954.1  protein Z-dependent protease inhibitor isoform X2

    UniProtKB/Swiss-Prot
    A5Z2A5, Q6UWX9, Q86U20, Q9UK55

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060938.1 Alternate T2T-CHM13v2.0

    Range
    88507743..88520553 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

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

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

Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q9UK55.1 GenPept UniProtKB/Swiss-Prot:Q9UK55

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