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Q9UK55

- ZPI_HUMAN

UniProt

Q9UK55 - ZPI_HUMAN

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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

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
Complete GO annotation...

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
  2. extracellular vesicular exosome Source: UniProtKB
Complete GO annotation...

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

BgeeiQ9UK55.
CleanExiHS_SERPINA10.
ExpressionAtlasiQ9UK55. baseline and differential.
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 – 9332Combined sources
Beta strandi98 – 1003Combined sources
Helixi102 – 11312Combined sources
Helixi119 – 1279Combined sources
Helixi137 – 1393Combined sources
Helixi140 – 15415Combined sources
Helixi156 – 1583Combined sources
Beta strandi160 – 16910Combined sources
Helixi176 – 18611Combined sources
Beta strandi189 – 1935Combined sources
Beta strandi195 – 1973Combined sources
Helixi198 – 21215Combined sources
Turni213 – 2153Combined sources
Beta strandi216 – 2183Combined sources
Beta strandi228 – 24417Combined sources
Helixi248 – 2503Combined sources
Beta strandi252 – 26110Combined sources
Beta strandi263 – 28018Combined sources
Turni281 – 2844Combined sources
Beta strandi285 – 2928Combined sources
Beta strandi295 – 30410Combined sources
Beta strandi305 – 3073Combined sources
Helixi312 – 3143Combined sources
Helixi318 – 3269Combined sources
Beta strandi329 – 33810Combined sources
Beta strandi340 – 3478Combined sources
Helixi349 – 3546Combined sources
Helixi359 – 3613Combined sources
Turni368 – 3703Combined sources
Beta strandi371 – 3733Combined sources
Beta strandi378 – 39013Combined sources
Beta strandi392 – 40615Combined sources
Beta strandi413 – 4153Combined sources
Beta strandi420 – 4267Combined sources
Turni427 – 4304Combined sources
Beta strandi431 – 4399Combined sources

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.
GeneTreeiENSGT00760000118839.
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

        10         20         30         40         50
MKVVPSLLLS VLLAQVWLVP GLAPSPQSPE TPAPQNQTSR VVQAPKEEEE
60 70 80 90 100
DEQEASEEKA SEEEKAWLMA SRQQLAKETS NFGFSLLRKI SMRHDGNMVF
110 120 130 140 150
SPFGMSLAMT GLMLGATGPT ETQIKRGLHL QALKPTKPGL LPSLFKGLRE
160 170 180 190 200
TLSRNLELGL TQGSFAFIHK DFDVKETFFN LSKRYFDTEC VPMNFRNASQ
210 220 230 240 250
AKRLMNHYIN KETRGKIPKL FDEINPETKL ILVDYILFKG KWLTPFDPVF
260 270 280 290 300
TEVDTFHLDK YKTIKVPMMY GAGKFASTFD KNFRCHVLKL PYQGNATMLV
310 320 330 340 350
VLMEKMGDHL ALEDYLTTDL VETWLRNMKT RNMEVFFPKF KLDQKYEMHE
360 370 380 390 400
LLRQMGIRRI FSPFADLSEL SATGRNLQVS RVLQRTVIEV DERGTEAVAG
410 420 430 440
ILSEITAYSM PPVIKVDRPF HFMIYEETSG MLLFLGRVVN PTLL
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.
GeneTreei ENSGT00760000118839.
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

Bgeei Q9UK55.
CleanExi HS_SERPINA10.
ExpressionAtlasi Q9UK55. baseline and differential.
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

« Hide 'large scale' publications
  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 29, 2014
This is version 138 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