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Protein

Alpha-2-antiplasmin

Gene

SERPINF2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Serine protease inhibitor. The major targets of this inhibitor are plasmin and trypsin, but it also inactivates matriptase-3/TMPRSS7 and chymotrypsin.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei403 – 404Reactive bond for plasmin2
Sitei404 – 405Reactive bond for chymotrypsin2

GO - Molecular functioni

  • endopeptidase inhibitor activity Source: ProtInc
  • protease binding Source: UniProtKB
  • protein homodimerization activity Source: BHF-UCL
  • serine-type endopeptidase inhibitor activity Source: BHF-UCL

GO - Biological processi

Keywordsi

Molecular functionProtease inhibitor, Serine protease inhibitor
Biological processAcute phase

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation
R-HSA-75205 Dissolution of Fibrin Clot

Protein family/group databases

MEROPSiI04.023

Names & Taxonomyi

Protein namesi
Recommended name:
Alpha-2-antiplasmin
Short name:
Alpha-2-AP
Alternative name(s):
Alpha-2-plasmin inhibitor
Short name:
Alpha-2-PI
Serpin F2
Gene namesi
Name:SERPINF2
Synonyms:AAP, PLI
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

EuPathDBiHostDB:ENSG00000167711.13
HGNCiHGNC:9075 SERPINF2
MIMi613168 gene
neXtProtiNX_P08697

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Involvement in diseasei

Alpha-2-plasmin inhibitor deficiency (APLID)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive disorder resulting in severe hemorrhagic diathesis.
See also OMIM:262850
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_013254176Missing in APLID; variant Okinawa; probably blocks intracellular transport of alpha-2-plasmin inhibitor. 1 Publication1
Natural variantiVAR_013255411V → M in APLID. 1 PublicationCorresponds to variant dbSNP:rs121965062EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi5345
MalaCardsiSERPINF2
MIMi262850 phenotype
OpenTargetsiENSG00000167711
Orphaneti79 Congenital alpha2-antiplasmin deficiency
PharmGKBiPA35522

Chemistry databases

DrugBankiDB08888 Ocriplasmin

Polymorphism and mutation databases

BioMutaiSERPINF2
DMDMi112907

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 272 PublicationsAdd BLAST27
PropeptideiPRO_000003251128 – 394 PublicationsAdd BLAST12
ChainiPRO_000003251240 – 491Alpha-2-antiplasminAdd BLAST452

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki41Isoglutamyl lysine isopeptide (Gln-Lys) (interchain with K-322 in alpha-fibrinogen)
Disulfide bondi70 ↔ 1431 Publication
Glycosylationi126N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi295N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi309N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi316N-linked (GlcNAc...) asparagineSequence analysis1
Modified residuei484Sulfotyrosine1 Publication1

Post-translational modificationi

Proteolytically cleaved at Pro-39 by both the prolyl endopeptidase FAP form and antiplasmin-cleaving enzyme FAP soluble form to generate mature alpha-2-antiplasmin.2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei39 – 40Cleavage; by prolyl endopeptidase FAP, antiplasmin-cleaving enzyme FAP soluble form2 Publications2

Keywords - PTMi

Disulfide bond, Glycoprotein, Isopeptide bond, Sulfation

Proteomic databases

MaxQBiP08697
PaxDbiP08697
PeptideAtlasiP08697
PRIDEiP08697
ProteomicsDBi52156
52157 [P08697-2]

2D gel databases

SWISS-2DPAGEiP08697

PTM databases

GlyConnecti1002
iPTMnetiP08697
PhosphoSitePlusiP08697

Miscellaneous databases

PMAP-CutDBiP08697

Expressioni

Tissue specificityi

Expressed by the liver and secreted in plasma.

Gene expression databases

BgeeiENSG00000167711 Expressed in 108 organ(s), highest expression level in right lobe of liver
CleanExiHS_SERPINF2
ExpressionAtlasiP08697 baseline and differential
GenevisibleiP08697 HS

Organism-specific databases

HPAiCAB024863
HPA001885

Interactioni

Subunit structurei

Forms protease inhibiting heterodimer with TMPRSS7.

GO - Molecular functioni

Protein-protein interaction databases

BioGridi111360, 10 interactors
IntActiP08697, 2 interactors
STRINGi9606.ENSP00000321853

Structurei

3D structure databases

ProteinModelPortaliP08697
SMRiP08697
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the serpin family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG2392 Eukaryota
COG4826 LUCA
GeneTreeiENSGT00930000150820
HOGENOMiHOG000231761
HOVERGENiHBG000043
InParanoidiP08697
KOiK03983
OMAiQGFWRSK
OrthoDBiEOG091G066U
PhylomeDBiP08697
TreeFamiTF317350

Family and domain databases

CDDicd02053 alpha2AP, 1 hit
InterProiView protein in InterPro
IPR033833 Alpha2AP
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
PANTHERiPTHR11461 PTHR11461, 1 hit
PfamiView protein in Pfam
PF00079 Serpin, 1 hit
SMARTiView protein in SMART
SM00093 SERPIN, 1 hit
SUPFAMiSSF56574 SSF56574, 1 hit
PROSITEiView protein in PROSITE
PS00284 SERPIN, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 5 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P08697-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MALLWGLLVL SWSCLQGPCS VFSPVSAMEP LGRQLTSGPN QEQVSPLTLL
60 70 80 90 100
KLGNQEPGGQ TALKSPPGVC SRDPTPEQTH RLARAMMAFT ADLFSLVAQT
110 120 130 140 150
STCPNLILSP LSVALALSHL ALGAQNHTLQ RLQQVLHAGS GPCLPHLLSR
160 170 180 190 200
LCQDLGPGAF RLAARMYLQK GFPIKEDFLE QSEQLFGAKP VSLTGKQEDD
210 220 230 240 250
LANINQWVKE ATEGKIQEFL SGLPEDTVLL LLNAIHFQGF WRNKFDPSLT
260 270 280 290 300
QRDSFHLDEQ FTVPVEMMQA RTYPLRWFLL EQPEIQVAHF PFKNNMSFVV
310 320 330 340 350
LVPTHFEWNV SQVLANLSWD TLHPPLVWER PTKVRLPKLY LKHQMDLVAT
360 370 380 390 400
LSQLGLQELF QAPDLRGISE QSLVVSGVQH QSTLELSEVG VEAAAATSIA
410 420 430 440 450
MSRMSLSSFS VNRPFLFFIF EDTTGLPLFV GSVRNPNPSA PRELKEQQDS
460 470 480 490
PGNKDFLQSL KGFPRGDKLF GPDLKLVPPM EEDYPQFGSP K
Length:491
Mass (Da):54,566
Last modified:November 1, 1990 - v3
Checksum:i385A1C90E91A63CB
GO
Isoform 2 (identifier: P08697-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     56-119: Missing.
     120-122: LAL → VQP

Show »
Length:427
Mass (Da):47,907
Checksum:i1A5190EC32B5350A
GO

Computationally mapped potential isoform sequencesi

There are 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A0G2JPA8A0A0G2JPA8_HUMAN
Alpha-2-antiplasmin
SERPINF2
427Annotation score:
C9JMH6C9JMH6_HUMAN
Alpha-2-antiplasmin
SERPINF2
255Annotation score:
C9JPV4C9JPV4_HUMAN
Alpha-2-antiplasmin
SERPINF2
264Annotation score:
A0A0J9YY65A0A0J9YY65_HUMAN
Alpha-2-antiplasmin
SERPINF2
255Annotation score:
A0A0J9YWQ3A0A0J9YWQ3_HUMAN
Alpha-2-antiplasmin
SERPINF2
264Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti49L → G AA sequence (PubMed:2440681).Curated1
Sequence conflicti105N → D AA sequence (PubMed:2440681).Curated1
Sequence conflicti289H → D in AAA35543 (PubMed:2433286).Curated1
Sequence conflicti408S → G AA sequence (PubMed:2440681).Curated1
Sequence conflicti455D → N AA sequence (PubMed:2440681).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0479512A → V1 PublicationCorresponds to variant dbSNP:rs2070862EnsemblClinVar.1
Natural variantiVAR_01325227A → V1 Publication1
Natural variantiVAR_01325333R → W1 PublicationCorresponds to variant dbSNP:rs2070863EnsemblClinVar.1
Natural variantiVAR_05195698A → G. Corresponds to variant dbSNP:rs36021516Ensembl.1
Natural variantiVAR_013254176Missing in APLID; variant Okinawa; probably blocks intracellular transport of alpha-2-plasmin inhibitor. 1 Publication1
Natural variantiVAR_013255411V → M in APLID. 1 PublicationCorresponds to variant dbSNP:rs121965062EnsemblClinVar.1
Natural variantiVAR_013256434R → K1 PublicationCorresponds to variant dbSNP:rs1057335EnsemblClinVar.1
Natural variantiVAR_061792451P → S. Corresponds to variant dbSNP:rs57360598Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04383356 – 119Missing in isoform 2. 1 PublicationAdd BLAST64
Alternative sequenceiVSP_043834120 – 122LAL → VQP in isoform 2. 1 Publication3

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D00174 mRNA Translation: BAA00124.1
M20786
, M20782, M20783, M20784, M20785 Genomic DNA Translation: AAA51554.1
AK303763 mRNA Translation: BAG64729.1
AC130343 Genomic DNA No translation available.
BC031592 mRNA Translation: AAH31592.1
J02654 mRNA Translation: AAA35543.1
D00116 mRNA Translation: BAA00070.1
CCDSiCCDS11011.1 [P08697-1]
CCDS54064.1 [P08697-2]
PIRiA31402 ITHUA2
RefSeqiNP_000925.2, NM_000934.3 [P08697-1]
NP_001159392.1, NM_001165920.1 [P08697-1]
NP_001159393.1, NM_001165921.1 [P08697-2]
UniGeneiHs.159509

Genome annotation databases

EnsembliENST00000324015; ENSP00000321853; ENSG00000167711 [P08697-1]
ENST00000382061; ENSP00000371493; ENSG00000167711 [P08697-1]
ENST00000450523; ENSP00000403877; ENSG00000167711 [P08697-2]
ENST00000618883; ENSP00000479005; ENSG00000276838
ENST00000622842; ENSP00000481874; ENSG00000276838
GeneIDi5345
KEGGihsa:5345
UCSCiuc002ftk.1 human [P08697-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D00174 mRNA Translation: BAA00124.1
M20786
, M20782, M20783, M20784, M20785 Genomic DNA Translation: AAA51554.1
AK303763 mRNA Translation: BAG64729.1
AC130343 Genomic DNA No translation available.
BC031592 mRNA Translation: AAH31592.1
J02654 mRNA Translation: AAA35543.1
D00116 mRNA Translation: BAA00070.1
CCDSiCCDS11011.1 [P08697-1]
CCDS54064.1 [P08697-2]
PIRiA31402 ITHUA2
RefSeqiNP_000925.2, NM_000934.3 [P08697-1]
NP_001159392.1, NM_001165920.1 [P08697-1]
NP_001159393.1, NM_001165921.1 [P08697-2]
UniGeneiHs.159509

3D structure databases

ProteinModelPortaliP08697
SMRiP08697
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111360, 10 interactors
IntActiP08697, 2 interactors
STRINGi9606.ENSP00000321853

Chemistry databases

DrugBankiDB08888 Ocriplasmin

Protein family/group databases

MEROPSiI04.023

PTM databases

GlyConnecti1002
iPTMnetiP08697
PhosphoSitePlusiP08697

Polymorphism and mutation databases

BioMutaiSERPINF2
DMDMi112907

2D gel databases

SWISS-2DPAGEiP08697

Proteomic databases

MaxQBiP08697
PaxDbiP08697
PeptideAtlasiP08697
PRIDEiP08697
ProteomicsDBi52156
52157 [P08697-2]

Protocols and materials databases

DNASUi5345
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000324015; ENSP00000321853; ENSG00000167711 [P08697-1]
ENST00000382061; ENSP00000371493; ENSG00000167711 [P08697-1]
ENST00000450523; ENSP00000403877; ENSG00000167711 [P08697-2]
ENST00000618883; ENSP00000479005; ENSG00000276838
ENST00000622842; ENSP00000481874; ENSG00000276838
GeneIDi5345
KEGGihsa:5345
UCSCiuc002ftk.1 human [P08697-1]

Organism-specific databases

CTDi5345
DisGeNETi5345
EuPathDBiHostDB:ENSG00000167711.13
GeneCardsiSERPINF2
H-InvDBiHIX0013407
HGNCiHGNC:9075 SERPINF2
HPAiCAB024863
HPA001885
MalaCardsiSERPINF2
MIMi262850 phenotype
613168 gene
neXtProtiNX_P08697
OpenTargetsiENSG00000167711
Orphaneti79 Congenital alpha2-antiplasmin deficiency
PharmGKBiPA35522
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2392 Eukaryota
COG4826 LUCA
GeneTreeiENSGT00930000150820
HOGENOMiHOG000231761
HOVERGENiHBG000043
InParanoidiP08697
KOiK03983
OMAiQGFWRSK
OrthoDBiEOG091G066U
PhylomeDBiP08697
TreeFamiTF317350

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation
R-HSA-75205 Dissolution of Fibrin Clot

Miscellaneous databases

ChiTaRSiSERPINF2 human
GeneWikiiAlpha_2-antiplasmin
GenomeRNAii5345
PMAP-CutDBiP08697
PROiPR:P08697
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000167711 Expressed in 108 organ(s), highest expression level in right lobe of liver
CleanExiHS_SERPINF2
ExpressionAtlasiP08697 baseline and differential
GenevisibleiP08697 HS

Family and domain databases

CDDicd02053 alpha2AP, 1 hit
InterProiView protein in InterPro
IPR033833 Alpha2AP
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
PANTHERiPTHR11461 PTHR11461, 1 hit
PfamiView protein in Pfam
PF00079 Serpin, 1 hit
SMARTiView protein in SMART
SM00093 SERPIN, 1 hit
SUPFAMiSSF56574 SSF56574, 1 hit
PROSITEiView protein in PROSITE
PS00284 SERPIN, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiA2AP_HUMAN
AccessioniPrimary (citable) accession number: P08697
Secondary accession number(s): B4E1B7
, Q8N5U7, Q9UCG2, Q9UCG3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1988
Last sequence update: November 1, 1990
Last modified: November 7, 2018
This is version 197 of the entry and version 3 of the sequence. See 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

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 17
    Human chromosome 17: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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