Reviewed,
UniProtKB/Swiss-Prot P08697 (A2AP_HUMAN)
Last modified
November 25, 2008.
Version 103.
History...
Clusters with 100%,
90%,
50% identity |
Documents (5) |
Third-party data |
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Names and origin
| Protein names | Recommended name: Alpha-2-antiplasmin Short name=Alpha-2-AP Alternative name(s): Alpha-2-plasmin inhibitor Short name=Alpha-2-PI | ||||
| Gene names |
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| Organism | Homo sapiens (Human) | ||||
| Taxonomic identifier | 9606 [NCBI] | ||||
| Taxonomic lineage | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Protein attributes
| Sequence length | 491 AA. |
| Sequence status | Complete. |
| Sequence processing | The displayed sequence is further processed into a mature form. |
| Protein existence | Evidence at protein level. |
General annotation (Comments)
| Function | The major targets of this inhibitor are plasmin and trypsin, but it also inactivates chymotrypsin. |
| Subcellular location | |
| Tissue specificity | Expressed by the liver and secreted in plasma. |
| Involvement in disease | Defects in SERPINF2 are the cause of alpha-2-plasmin inhibitor deficiency (APLID) [MIM:262850]. APLID is an autosomal recessive disorder resulting in severe hemorrhagic diathesis. |
| Sequence similarities | Belongs to the serpin family. |
Ontologies
Keywords | |
|---|---|
| Biological process | Acute phase |
| Cellular component | Secreted |
| Coding sequence diversity | Polymorphism |
| Disease | Disease mutation |
| Domain | Signal |
| Molecular function | Protease inhibitor Serine protease inhibitor |
| PTM | Glycoprotein Sulfation |
| Technical term | Direct protein sequencing |
Gene Ontology (GO) | |
| Biological process | acute-phase response Inferred from electronic annotation. Source: UniProtKB-KW |
| Cellular component | extracellular region Non-traceable author statement. Source: UniProtKB nucleusInferred from direct assay. Source: HPA platelet alpha granule lumenInferred from Experiment. Source: Reactome |
| Molecular function | protein binding Inferred from physical interaction. Source: UniProtKB serine-type endopeptidase inhibitor activity Ref.4Non-traceable author statement. Source: UniProtKB |
| Complete GO annotation... | |
Sequence annotation (Features)
| Feature key | Position(s) | Length | Description | Graphical view | Feature identifier | ||||||
Molecule processing | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Signal peptide | 1 – 27 | 27 | |||||||||
| Propeptide | 28 – 39 | 12 | PRO_0000032511 | ||||||||
| Chain | 40 – 491 | 452 | Alpha-2-antiplasmin | PRO_0000032512 | |||||||
Sites | |||||||||||
| Site | 403 – 404 | 2 | Reactive bond for plasmin | ||||||||
| Site | 404 – 405 | 2 | Reactive bond for chymotrypsin | ||||||||
Amino acid modifications | |||||||||||
| Modified residue | 484 | 1 | Sulfotyrosine | ||||||||
| Glycosylation | 126 | 1 | N-linked (GlcNAc...) | ||||||||
| Glycosylation | 295 | 1 | N-linked (GlcNAc...) | ||||||||
| Glycosylation | 309 | 1 | N-linked (GlcNAc...) | ||||||||
| Glycosylation | 316 | 1 | N-linked (GlcNAc...) Potential | ||||||||
| Disulfide bond | 70 ↔ 143 | ||||||||||
| Cross-link | 41 | Isoglutamyl lysine isopeptide (Gln-Lys) (interchain with K-322 in alpha-fibrinogen) | |||||||||
Natural variations | |||||||||||
| Natural variant | 27 | 1 | A → V | VAR_013252 | |||||||
| Natural variant | 33 | 1 | R → W | VAR_013253 | |||||||
| Natural variant | 176 | 1 | Missing in APLID; variant Okinawa; probably blocks intracellular transport of alpha-2-plasmin inhibitor. | VAR_013254 | |||||||
| Natural variant | 411 | 1 | V → M in APLID. | VAR_013255 | |||||||
| Natural variant | 434 | 1 | R → K | VAR_013256 | |||||||
Experimental info | |||||||||||
| Sequence conflict | 49 | 1 | L → G AA sequence Ref.6 | ||||||||
| Sequence conflict | 105 | 1 | N → D AA sequence Ref.6 | ||||||||
| Sequence conflict | 289 | 1 | H → D in AAA35543. Ref.4 | ||||||||
| Sequence conflict | 408 | 1 | S → G AA sequence Ref.6 | ||||||||
| Sequence conflict | 455 | 1 | D → N AA sequence Ref.6 | ||||||||
Sequences
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References
| « Hide 'large scale' references | |
| [1] | "Structure of human alpha 2-plasmin inhibitor deduced from the cDNA sequence." Tone M., Kikuno R., Kume-Iwaki A., Hashimoto-Gotoh T. J. Biochem. 102:1033-1041(1987) [PubMed: 2830248] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Tissue: Liver. |
| [2] | "Organization of the human alpha 2-plasmin inhibitor gene." Hirosawa S., Nakamura Y., Miura O., Sumi Y., Aoki N. Proc. Natl. Acad. Sci. U.S.A. 85:6836-6840(1988) [PubMed: 3166140] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA]. |
| [3] | Erratum Hirosawa S., Nakamura Y., Miura O., Sumi Y., Aoki N. Proc. Natl. Acad. Sci. U.S.A. 86:1612-1613(1989) |
| [4] | "Primary structure of human alpha 2-antiplasmin, a serine protease inhibitor (serpin)." Holmes W.E., Nelles L., Lijnen H.R., Collen D. J. Biol. Chem. 262:1659-1664(1987) [PubMed: 2433286] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 4-491. |
| [5] | "Structure of the carboxyl-terminal half of human alpha 2-plasmin inhibitor deduced from that of cDNA." Sumi Y., Nakamura Y., Aoki N., Sakai M., Muramatsu M. J. Biochem. 100:1399-1402(1986) [PubMed: 3818581] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 218-491. |
| [6] | "Amino-acid sequence of human alpha 2-antiplasmin." Lijnen H.R., Holmes W.E., van Hoef B., Wiman B., Rodriguez H., Collen D. Eur. J. Biochem. 166:565-574(1987) [PubMed: 2440681] [Abstract] Cited for: PROTEIN SEQUENCE OF 40-491. |
| [7] | "Purification and characterization of human antiplasmin, the fast-acting plasmin inhibitor in plasma." Wiman B., Collen D. Eur. J. Biochem. 78:19-26(1977) [PubMed: 21075] [Abstract] Cited for: PROTEIN SEQUENCE OF 40-43. |
| [8] | "Bovine alpha 2-antiplasmin. N-terminal and reactive site sequence." Christensen S., Sottrup-Jensen L. FEBS Lett. 312:100-104(1992) [PubMed: 1385210] [Abstract] Cited for: PROTEIN SEQUENCE OF 28-52. Tissue: Plasma. |
| [9] | "Alpha-2-antiplasmin: a serpin with two separate but overlapping reactive sites." Potempa J., Shieh B.-H., Travis J. Science 241:699-700(1988) [PubMed: 2456616] [Abstract] Cited for: ACTIVE SITES. |
| [10] | "Assignment of a single disulphide bridge in human alpha2-antiplasmin: implications for the structural and functional properties." Christensen S., Valnickova Z., Thogersen I.B., Olsen E.H., Enghild J.J. Biochem. J. 323:847-852(1997) [PubMed: 9169621] [Abstract] Cited for: DISULFIDE BOND. |
| [11] | "Sulfation of a tyrosine residue in the plasmin-binding domain of alpha 2-antiplasmin." Hortin G., Fok K.F., Toren P.C., Strauss A.W. J. Biol. Chem. 262:3082-3085(1987) [PubMed: 2434496] [Abstract] Cited for: PROTEIN SEQUENCE OF 481-491, SULFATION. |
| [12] | "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: 16335952] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-126; ASN-295 AND ASN-309, MASS SPECTROMETRY. Tissue: Plasma. |
| [13] | "Hereditary alpha 2-plasmin inhibitor deficiency caused by a transport-deficient mutation (alpha 2-PI-Okinawa). Deletion of Glu137 by a trinucleotide deletion blocks intracellular transport." Miura O., Sugahara Y., Aoki N. J. Biol. Chem. 264:18213-18219(1989) [PubMed: 2572590] [Abstract] Cited for: VARIANT APLID GLU-176 DEL. |
| [14] | "A novel missense mutation in the human plasmin inhibitor (alpha2-antiplasmin) gene associated with a bleeding tendency." Lind B., Thorsen S. Br. J. Haematol. 107:317-322(1999) [PubMed: 10583218] [Abstract] Cited for: VARIANT APLID MET-411, VARIANTS VAL-27; TRP-33 AND LYS-434. |
| + | Additional computationally mapped references. |
Cross-references
Sequence databases | |
|---|---|
| D00116 mRNA. Translation: BAA00070.1. D00174 mRNA. Translation: BAA00124.1. M20786 M20785 Genomic DNA. Translation: AAA51554.1. J02654 mRNA. Translation: AAA35543.1. | |
| PIR | ITHUA2. A31402. |
| RefSeq | NP_000925.2. |
| UniGene | Hs.159509 |
3D structure databases | |
| HSSP | HSSP built from PDB template 1ANT based on UniProtKB P01008. |
| ModBase | Search... |
Protein family/group databases | |
| MEROPS | I04.023. |
2-D gel databases | |
| SWISS-2DPAGE | P08697. |
Genome annotation databases | |
| Ensembl | ENSG00000167711. Homo sapiens. [Contig view] |
| GeneID | 5345. |
| KEGG | hsa:5345. |
Organism-specific databases | |
| H-InvDB | HIX0013407. |
| HGNC | HGNC:9075. SERPINF2. |
| HPA | HPA001885. |
| MIM | 262850. gene+phenotype. |
| PharmGKB | PA35522. |
| GenAtlas | Search... |
| GeneCards | Search... |
Phylogenomic databases | |
| HOGENOM | P08697. |
| HOVERGEN | P08697. |
Enzyme and pathway databases | |
| Reactome | REACT_604. Hemostasis. |
Gene expression databases | |
| ArrayExpress | P08697. |
| CleanEx | HS_SERPINF2. |
Family and domain databases | |
| InterPro | IPR000215. Protease_inhib_I4_serpin. [Graphical view] |
| PANTHER | PTHR11461. Prot_inh_serpin. 1 hit. |
| Pfam | PF00079. Serpin. 1 hit. [Graphical view] |
| SMART | SM00093. SERPIN. 1 hit. [Graphical view] |
| PROSITE | PS00284. SERPIN. 1 hit. [Graphical view] |
| ProtoNet | Search... |
Other Resources | |
| DrugBank | DB00086. Streptokinase. |
| NextBio | 20714. |
| SOURCE | Search... |
Entry information
| Entry name | A2AP_HUMAN | ||||||||
| Accession | Primary (citable) accession number: P08697 | ||||||||
| Entry history |
| ||||||||
| Entry status | Reviewed (UniProtKB/Swiss-Prot) | ||||||||
| Annotation project | HPI (Human Proteome Initiative) | ||||||||
Relevant documents
| Human chromosome 17 Human chromosome 17: entries, gene names and cross-references to MIM |
| Human entries with polymorphisms or disease mutations List of human entries with polymorphisms or disease mutations |
| Human polymorphisms and disease mutations Index of human polymorphisms and disease mutations |
| MIM cross-references Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot |
| SIMILARITY comments Index of protein domains and families |

Clusters with


