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Q9UK55

- ZPI_HUMAN

UniProt

Q9UK55 - ZPI_HUMAN

Protein

Protein Z-dependent protease inhibitor

Gene

SERPINA10

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 137 (01 Oct 2014)
      Sequence version 1 (01 May 2000)
      Previous versions | rss
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    Functioni

    Inhibits activity of the coagulation protease factor Xa in the presence of PROZ, calcium and phospholipids. Also inhibits factor XIa in the absence of cofactors.1 Publication

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sitei261 – 2611Essential for interaction with PROZBy similarity
    Sitei314 – 3141Essential for interaction with PROZBy similarity
    Sitei408 – 4092Reactive bondBy similarity

    GO - Molecular functioni

    1. heparin binding Source: UniProtKB-KW
    2. serine-type endopeptidase inhibitor activity Source: RefGenome

    GO - Biological processi

    1. blood coagulation Source: UniProtKB-KW
    2. negative regulation of endopeptidase activity Source: RefGenome
    3. regulation of proteolysis Source: RefGenome

    Keywords - Molecular functioni

    Protease inhibitor, Serine protease inhibitor

    Keywords - Biological processi

    Blood coagulation, Hemostasis

    Keywords - Ligandi

    Heparin-binding

    Protein family/group databases

    MEROPSiI04.005.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Protein Z-dependent protease inhibitor
    Short name:
    PZ-dependent protease inhibitor
    Short name:
    PZI
    Alternative name(s):
    Serpin A10
    Gene namesi
    Name:SERPINA10
    Synonyms:ZPI
    ORF Names:UNQ707/PRO1358
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 14

    Organism-specific databases

    HGNCiHGNC:15996. SERPINA10.

    Subcellular locationi

    GO - Cellular componenti

    1. extracellular space Source: InterPro

    Keywords - Cellular componenti

    Secreted

    Pathology & Biotechi

    Mutagenesis

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Mutagenesisi408 – 4081Y → A: Loss of inhibitory activity. 1 Publication

    Organism-specific databases

    MIMi605271. gene+phenotype.
    PharmGKBiPA38078.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Signal peptidei1 – 21211 PublicationAdd
    BLAST
    Chaini22 – 444423Protein Z-dependent protease inhibitorPRO_0000032482Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Glycosylationi36 – 361N-linked (GlcNAc...)1 Publication
    Glycosylationi180 – 1801N-linked (GlcNAc...)1 Publication
    Glycosylationi197 – 1971N-linked (GlcNAc...)1 Publication
    Glycosylationi295 – 2951N-linked (GlcNAc...)2 Publications

    Post-translational modificationi

    Phosphorylation sites are present in the extracellular medium.

    Keywords - PTMi

    Glycoprotein, Phosphoprotein

    Proteomic databases

    PaxDbiQ9UK55.
    PRIDEiQ9UK55.

    PTM databases

    PhosphoSiteiQ9UK55.

    Expressioni

    Tissue specificityi

    Expressed by the liver and secreted in plasma.

    Gene expression databases

    ArrayExpressiQ9UK55.
    BgeeiQ9UK55.
    CleanExiHS_SERPINA10.
    GenevestigatoriQ9UK55.

    Organism-specific databases

    HPAiHPA048739.

    Interactioni

    Subunit structurei

    Interacts with PROZ.3 Publications

    Protein-protein interaction databases

    BioGridi119339. 2 interactions.
    IntActiQ9UK55. 1 interaction.
    STRINGi9606.ENSP00000261994.

    Structurei

    Secondary structure

    1
    444
    Legend: HelixTurnBeta strand
    Show more details
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Helixi62 – 9332
    Beta strandi98 – 1003
    Helixi102 – 11312
    Helixi119 – 1279
    Helixi137 – 1393
    Helixi140 – 15415
    Helixi156 – 1583
    Beta strandi160 – 16910
    Helixi176 – 18611
    Beta strandi189 – 1935
    Beta strandi195 – 1973
    Helixi198 – 21215
    Turni213 – 2153
    Beta strandi216 – 2183
    Beta strandi228 – 24417
    Helixi248 – 2503
    Beta strandi252 – 26110
    Beta strandi263 – 28018
    Turni281 – 2844
    Beta strandi285 – 2928
    Beta strandi295 – 30410
    Beta strandi305 – 3073
    Helixi312 – 3143
    Helixi318 – 3269
    Beta strandi329 – 33810
    Beta strandi340 – 3478
    Helixi349 – 3546
    Helixi359 – 3613
    Turni368 – 3703
    Beta strandi371 – 3733
    Beta strandi378 – 39013
    Beta strandi392 – 40615
    Beta strandi413 – 4153
    Beta strandi420 – 4267
    Turni427 – 4304
    Beta strandi431 – 4399

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    EntryMethodResolution (Å)ChainPositionsPDBsum
    3F1SX-ray2.30A60-444[»]
    3H5CX-ray3.26A22-444[»]
    4AFXX-ray2.09A23-408[»]
    B409-444[»]
    4AJUX-ray2.65A23-408[»]
    B409-444[»]
    ProteinModelPortaliQ9UK55.
    SMRiQ9UK55. Positions 26-444.
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiQ9UK55.

    Family & Domainsi

    Region

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Regioni136 – 15318Heparin-bindingAdd
    BLAST

    Sequence similaritiesi

    Belongs to the serpin family.Curated

    Keywords - Domaini

    Signal

    Phylogenomic databases

    eggNOGiCOG4826.
    HOGENOMiHOG000238519.
    HOVERGENiHBG101138.
    InParanoidiQ9UK55.
    PhylomeDBiQ9UK55.
    TreeFamiTF343094.

    Family and domain databases

    InterProiIPR023796. Serpin_dom.
    IPR000215. Serpin_fam.
    [Graphical view]
    PANTHERiPTHR11461. PTHR11461. 1 hit.
    PfamiPF00079. Serpin. 1 hit.
    [Graphical view]
    SMARTiSM00093. SERPIN. 1 hit.
    [Graphical view]
    SUPFAMiSSF56574. SSF56574. 2 hits.

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    Q9UK55-1 [UniParc]FASTAAdd to Basket

    « Hide

    MKVVPSLLLS VLLAQVWLVP GLAPSPQSPE TPAPQNQTSR VVQAPKEEEE    50
    DEQEASEEKA SEEEKAWLMA SRQQLAKETS NFGFSLLRKI SMRHDGNMVF 100
    SPFGMSLAMT GLMLGATGPT ETQIKRGLHL QALKPTKPGL LPSLFKGLRE 150
    TLSRNLELGL TQGSFAFIHK DFDVKETFFN LSKRYFDTEC VPMNFRNASQ 200
    AKRLMNHYIN KETRGKIPKL FDEINPETKL ILVDYILFKG KWLTPFDPVF 250
    TEVDTFHLDK YKTIKVPMMY GAGKFASTFD KNFRCHVLKL PYQGNATMLV 300
    VLMEKMGDHL ALEDYLTTDL VETWLRNMKT RNMEVFFPKF KLDQKYEMHE 350
    LLRQMGIRRI FSPFADLSEL SATGRNLQVS RVLQRTVIEV DERGTEAVAG 400
    ILSEITAYSM PPVIKVDRPF HFMIYEETSG MLLFLGRVVN PTLL 444
    Length:444
    Mass (Da):50,707
    Last modified:May 1, 2000 - v1
    Checksum:i06BD47C97BBDD700
    GO

    Sequence cautioni

    The sequence CAD62339.1 differs from that shown. Reason: Erroneous initiation.

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti46 – 461K → R.2 Publications
    Corresponds to variant rs941590 [ dbSNP | Ensembl ].
    VAR_020325
    Natural varianti61 – 611S → G.2 Publications
    Corresponds to variant rs941591 [ dbSNP | Ensembl ].
    VAR_020326
    Natural varianti139 – 1391G → R.1 Publication
    Corresponds to variant rs56137907 [ dbSNP | Ensembl ].
    VAR_038833
    Natural varianti158 – 1581L → Q.
    Corresponds to variant rs2232699 [ dbSNP | Ensembl ].
    VAR_051940
    Natural varianti161 – 1611T → S.2 Publications
    Corresponds to variant rs2232700 [ dbSNP | Ensembl ].
    VAR_020327
    Natural varianti196 – 1961R → H.
    Corresponds to variant rs2232701 [ dbSNP | Ensembl ].
    VAR_051941
    Natural varianti271 – 2711G → S.1 Publication
    Corresponds to variant rs2232708 [ dbSNP | Ensembl ].
    VAR_038834
    Natural varianti384 – 3841Q → P.1 Publication
    VAR_038835
    Natural varianti384 – 3841Q → R.1 Publication
    Corresponds to variant rs2232710 [ dbSNP | Ensembl ].
    VAR_051942
    Natural varianti420 – 4201F → L.1 Publication
    VAR_070192

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF181467 mRNA. Translation: AAD53962.1.
    AY358597 mRNA. Translation: AAQ88960.1.
    BX248011 mRNA. Translation: CAD62339.1. Different initiation.
    EF621762 Genomic DNA. Translation: ABR09269.1.
    BC022261 mRNA. Translation: AAH22261.1.
    CCDSiCCDS9923.1.
    RefSeqiNP_001094077.1. NM_001100607.2.
    NP_057270.1. NM_016186.2.
    XP_005267790.1. XM_005267733.2.
    XP_006725227.1. XM_006725164.1.
    UniGeneiHs.118620.

    Genome annotation databases

    EnsembliENST00000261994; ENSP00000261994; ENSG00000140093.
    ENST00000393096; ENSP00000376809; ENSG00000140093.
    ENST00000554173; ENSP00000450971; ENSG00000140093.
    GeneIDi51156.
    KEGGihsa:51156.
    UCSCiuc001yct.3. human.

    Polymorphism databases

    DMDMi12585541.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Web resourcesi

    SeattleSNPs

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF181467 mRNA. Translation: AAD53962.1 .
    AY358597 mRNA. Translation: AAQ88960.1 .
    BX248011 mRNA. Translation: CAD62339.1 . Different initiation.
    EF621762 Genomic DNA. Translation: ABR09269.1 .
    BC022261 mRNA. Translation: AAH22261.1 .
    CCDSi CCDS9923.1.
    RefSeqi NP_001094077.1. NM_001100607.2.
    NP_057270.1. NM_016186.2.
    XP_005267790.1. XM_005267733.2.
    XP_006725227.1. XM_006725164.1.
    UniGenei Hs.118620.

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    Entry Method Resolution (Å) Chain Positions PDBsum
    3F1S X-ray 2.30 A 60-444 [» ]
    3H5C X-ray 3.26 A 22-444 [» ]
    4AFX X-ray 2.09 A 23-408 [» ]
    B 409-444 [» ]
    4AJU X-ray 2.65 A 23-408 [» ]
    B 409-444 [» ]
    ProteinModelPortali Q9UK55.
    SMRi Q9UK55. Positions 26-444.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 119339. 2 interactions.
    IntActi Q9UK55. 1 interaction.
    STRINGi 9606.ENSP00000261994.

    Protein family/group databases

    MEROPSi I04.005.

    PTM databases

    PhosphoSitei Q9UK55.

    Polymorphism databases

    DMDMi 12585541.

    Proteomic databases

    PaxDbi Q9UK55.
    PRIDEi Q9UK55.

    Protocols and materials databases

    DNASUi 51156.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000261994 ; ENSP00000261994 ; ENSG00000140093 .
    ENST00000393096 ; ENSP00000376809 ; ENSG00000140093 .
    ENST00000554173 ; ENSP00000450971 ; ENSG00000140093 .
    GeneIDi 51156.
    KEGGi hsa:51156.
    UCSCi uc001yct.3. human.

    Organism-specific databases

    CTDi 51156.
    GeneCardsi GC14M094749.
    HGNCi HGNC:15996. SERPINA10.
    HPAi HPA048739.
    MIMi 602455. gene.
    605271. gene+phenotype.
    neXtProti NX_Q9UK55.
    PharmGKBi PA38078.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG4826.
    HOGENOMi HOG000238519.
    HOVERGENi HBG101138.
    InParanoidi Q9UK55.
    PhylomeDBi Q9UK55.
    TreeFami TF343094.

    Miscellaneous databases

    EvolutionaryTracei Q9UK55.
    GenomeRNAii 51156.
    NextBioi 54061.
    PROi Q9UK55.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q9UK55.
    Bgeei Q9UK55.
    CleanExi HS_SERPINA10.
    Genevestigatori Q9UK55.

    Family and domain databases

    InterProi IPR023796. Serpin_dom.
    IPR000215. Serpin_fam.
    [Graphical view ]
    PANTHERi PTHR11461. PTHR11461. 1 hit.
    Pfami PF00079. Serpin. 1 hit.
    [Graphical view ]
    SMARTi SM00093. SERPIN. 1 hit.
    [Graphical view ]
    SUPFAMi SSF56574. SSF56574. 2 hits.
    ProtoNeti Search...

    Publicationsi

    1. "The protein Z-dependent protease inhibitor is a serpin."
      Han X., Huang Z.-F., Fiehler R., Broze G.J. Jr.
      Biochemistry 38:11073-11078(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], MUTAGENESIS OF TYR-408.
      Tissue: Liver.
    2. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANTS ARG-46; GLY-61; SER-161 AND ARG-384.
    3. "Full-length cDNA libraries and normalization."
      Li W.B., Gruber C., Jessee J., Polayes D.
      Submitted (FEB-2003) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Fetal liver.
    4. SeattleSNPs variation discovery resource
      Submitted (MAY-2007) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS ARG-46; GLY-61; ARG-139; SER-161; SER-271 AND PRO-384.
    5. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Liver.
    6. "Isolation of a protein Z-dependent plasma protease inhibitor."
      Han X., Fiehler R., Broze G.J. Jr.
      Proc. Natl. Acad. Sci. U.S.A. 95:9250-9255(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 22-33, CHARACTERIZATION.
      Tissue: Plasma.
    7. "Characterization of the protein Z-dependent protease inhibitor."
      Han X., Fiehler R., Broze G.J. Jr.
      Blood 96:3049-3055(2000) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION, INTERACTION WITH PROZ.
    8. "Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
      Liu T., Qian W.-J., Gritsenko M.A., Camp D.G. II, Monroe M.E., Moore R.J., Smith R.D.
      J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-180 AND ASN-295.
      Tissue: Plasma.
    9. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
      Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.
      J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-36.
      Tissue: Liver.
    10. "Characterization of the heparin-binding site of the protein z-dependent protease inhibitor."
      Yang L., Ding Q., Huang X., Olson S.T., Rezaie A.R.
      Biochemistry 51:4078-4085(2012) [PubMed] [Europe PMC] [Abstract]
      Cited for: HEPARIN-BINDING REGION.
    11. "Crystal structure of protein Z-dependent inhibitor complex shows how protein Z functions as a cofactor in the membrane inhibition of factor X."
      Wei Z., Yan Y., Carrell R.W., Zhou A.
      Blood 114:3662-3667(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: X-RAY CRYSTALLOGRAPHY (2.3 ANGSTROMS) OF 60-444 IN COMPLEX WITH PROZ.
    12. "Basis for the specificity and activation of the serpin protein Z-dependent proteinase inhibitor (ZPI) as an inhibitor of membrane-associated factor Xa."
      Huang X., Dementiev A., Olson S.T., Gettins P.G.
      J. Biol. Chem. 285:20399-20409(2010) [PubMed] [Europe PMC] [Abstract]
      Cited for: X-RAY CRYSTALLOGRAPHY (3.26 ANGSTROMS) OF 22-444 IN COMPLEX WITH PROZ, GLYCOSYLATION AT ASN-197 AND ASN-295, SUBUNIT.
    13. "Identification of mutations in SLC24A4, encoding a potassium-dependent sodium/calcium exchanger, as a cause of amelogenesis imperfecta."
      Parry D.A., Poulter J.A., Logan C.V., Brookes S.J., Jafri H., Ferguson C.H., Anwari B.M., Rashid Y., Zhao H., Johnson C.A., Inglehearn C.F., Mighell A.J.
      Am. J. Hum. Genet. 92:307-312(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT LEU-420.

    Entry informationi

    Entry nameiZPI_HUMAN
    AccessioniPrimary (citable) accession number: Q9UK55
    Secondary accession number(s): A5Z2A5, Q6UWX9, Q86U20
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: January 24, 2001
    Last sequence update: May 1, 2000
    Last modified: October 1, 2014
    This is version 137 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

    Miscellaneousi

    Miscellaneous

    Heparin acts as an important cofactor, producing 20 to 100-fold accelerations of SERPINA10 reactions with factor Xa and factor XIa.

    Keywords - Technical termi

    3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. Human chromosome 14
      Human chromosome 14: entries, gene names and cross-references to MIM
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    6. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3