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Entry version 243 (16 Oct 2019)
Sequence version 1 (21 Jul 1986)
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Protein

Antithrombin-III

Gene

SERPINC1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Most important serine protease inhibitor in plasma that regulates the blood coagulation cascade. AT-III inhibits thrombin, matriptase-3/TMPRSS7, as well as factors IXa, Xa and XIa. Its inhibitory activity is greatly enhanced in the presence of heparin.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei81Heparin1
Binding sitei161Heparin1
Binding sitei177Heparin1
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections (‘Function’, ‘PTM / Processing’, ‘Pathology and Biotech’) according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei425 – 426Reactive bond2

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionHeparin-binding, Protease inhibitor, Serine protease inhibitor
Biological processBlood coagulation, Hemostasis

Enzyme and pathway databases

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-140837 Intrinsic Pathway of Fibrin Clot Formation
R-HSA-140875 Common Pathway of Fibrin Clot Formation
R-HSA-381426 Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
R-HSA-8957275 Post-translational protein phosphorylation

SIGNOR Signaling Network Open Resource

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SIGNORi
P01008

Protein family/group databases

MEROPS protease database

More...
MEROPSi
I04.018

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Antithrombin-III
Short name:
ATIII
Alternative name(s):
Serpin C1
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:SERPINC1
Synonyms:AT3
ORF Names:PRO0309
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 1

Organism-specific databases

Human Gene Nomenclature Database

More...
HGNCi
HGNC:775 SERPINC1

Online Mendelian Inheritance in Man (OMIM)

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MIMi
107300 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P01008

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>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 & Seán O’Donoghue; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Antithrombin III deficiency (AT3D)38 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn important risk factor for hereditary thrombophilia, a hemostatic disorder characterized by a tendency to recurrent thrombosis. Antithrombin-III deficiency is classified into 4 types. Type I: characterized by a 50% decrease in antigenic and functional levels. Type II: has defects affecting the thrombin-binding domain. Type III: alteration of the heparin-binding domain. Plasma AT-III antigen levels are normal in type II and III. Type IV: consists of miscellaneous group of unclassifiable mutations.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_02745017Y → S in AT3D; type-I. 1 Publication1
Natural variantiVAR_01274823L → P in AT3D; type-I; does not undergo post-translational glycosylation. 2 PublicationsCorresponds to variant dbSNP:rs387906575EnsemblClinVar.1
Natural variantiVAR_02745132C → R in AT3D; type-I. 1 Publication1
Natural variantiVAR_00703339I → N in AT3D; type-II; Rouen-3; lack of heparin-binding properties. 1 PublicationCorresponds to variant dbSNP:rs121909558EnsemblClinVar.1
Natural variantiVAR_07119953C → F in AT3D. 1 Publication1
Natural variantiVAR_00703556R → C in AT3D; type-II; Rouen-4; lack of heparin-binding properties. 2 PublicationsCorresponds to variant dbSNP:rs28929469EnsemblClinVar.1
Natural variantiVAR_00703673P → L in AT3D; type-II; lacks heparin-binding ability. 4 PublicationsCorresponds to variant dbSNP:rs121909551EnsemblClinVar.1
Natural variantiVAR_00703779R → C in AT3D; Tours/Alger/Amiens/Toyama/Paris-1/Paris-2/Padua-2/Barcelona-2/Kumamoto/Omura/Sasebo; lacks heparin-binding ability. 3 PublicationsCorresponds to variant dbSNP:rs121909547EnsemblClinVar.1
Natural variantiVAR_00703879R → H in AT3D; type-II; Rouen-1/Padua-1/Bligny/Budapest-2; lack of heparin-binding properties. 2 PublicationsCorresponds to variant dbSNP:rs121909552EnsemblClinVar.1
Natural variantiVAR_00703979R → S in AT3D; type-II; Rouen-2; lack of heparin-binding properties. 1 PublicationCorresponds to variant dbSNP:rs121909547EnsemblClinVar.1
Natural variantiVAR_00704087Missing in AT3D; type-I. 1 Publication1
Natural variantiVAR_00704189R → C in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs147266200Ensembl.1
Natural variantiVAR_00704290F → L in AT3D; type-I; Budapest-6. 1 Publication1
Natural variantiVAR_02745295Y → C in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs907768931Ensembl.1
Natural variantiVAR_01231695Y → S in AT3D; type-I. 2 Publications1
Natural variantiVAR_02745398L → P in AT3D; type-I. 1 Publication1
Natural variantiVAR_007043108 – 109Missing in AT3D; type-I. 2
Natural variantiVAR_007044112P → T in AT3D; type-I. 2 Publications1
Natural variantiVAR_027454121M → K in AT3D; type-I. 1 Publication1
Natural variantiVAR_071200125G → D in AT3D. 1 Publication1
Natural variantiVAR_027455127C → R in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs121909573EnsemblClinVar.1
Natural variantiVAR_007045131L → F in AT3D; type-II; Budapest-3/Budapest-7. 2 PublicationsCorresponds to variant dbSNP:rs121909567EnsemblClinVar.1
Natural variantiVAR_007046131L → V in AT3D; type-II; Southport. 2 Publications1
Natural variantiVAR_007047133Q → K in AT3D; type I. 1 PublicationCorresponds to variant dbSNP:rs1411331203Ensembl.1
Natural variantiVAR_007048138 – 139Missing in AT3D; type-I. 1 Publication2
Natural variantiVAR_027456146K → E in AT3D; Dreux; complete loss af heparin binding. 1 PublicationCorresponds to variant dbSNP:rs1170430756Ensembl.1
Natural variantiVAR_007049148S → P in AT3D; type-II; Nagasaki; defective heparin binding associated with thrombosis. 2 PublicationsCorresponds to variant dbSNP:rs121909569EnsemblClinVar.1
Natural variantiVAR_007050150Q → P in AT3D; type-II; Vienna. 2 PublicationsCorresponds to variant dbSNP:rs765445413Ensembl.1
Natural variantiVAR_012749152 – 154Missing in AT3D; type-I. 1 Publication3
Natural variantiVAR_007051152H → Y in AT3D; type-I. 1 Publication1
Natural variantiVAR_007052153Missing in AT3D; type-I. 1
Natural variantiVAR_007053158L → P in AT3D; type-I. 1 Publication1
Natural variantiVAR_027457160C → Y in AT3D; type-I. 2 Publications1
Natural variantiVAR_007054161R → Q in AT3D; type-II; Geneva. 2 PublicationsCorresponds to variant dbSNP:rs121909563EnsemblClinVar.1
Natural variantiVAR_071201170S → P in AT3D. 1 Publication1
Natural variantiVAR_027458178L → H in AT3D; type-I. 1 Publication1
Natural variantiVAR_027459179F → L in AT3D; type-I. 1 Publication1
Natural variantiVAR_007056198Y → C in AT3D; type-I and -II; Whitechapel. 1 PublicationCorresponds to variant dbSNP:rs1425532034Ensembl.1
Natural variantiVAR_027460198Y → H in AT3D; type-I. 2 Publications1
Natural variantiVAR_027461214S → F in AT3D; type-I. 1 Publication1
Natural variantiVAR_007057214S → Y in AT3D; type-I. Corresponds to variant dbSNP:rs483352854EnsemblClinVar.1
Natural variantiVAR_071202218I → N in AT3D. 1 Publication1
Natural variantiVAR_027462218Missing in AT3D; type-I. 1 Publication1
Natural variantiVAR_007059219N → D in AT3D; type-II; Rouen-6; increases affinity for heparin. 2 PublicationsCorresponds to variant dbSNP:rs121909571EnsemblClinVar.1
Natural variantiVAR_007058219N → K in AT3D; type-II; Glasgow-3. 1 Publication1
Natural variantiVAR_027463223S → P in AT3D; type-I. 2 PublicationsCorresponds to variant dbSNP:rs121909572EnsemblClinVar.1
Natural variantiVAR_027464243T → I in AT3D; type-I. 1 Publication1
Natural variantiVAR_071203248V → G in AT3D. 1 Publication1
Natural variantiVAR_027465251I → T in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs1423630663Ensembl.1
Natural variantiVAR_027466257W → R in AT3D; type-I. 2 Publications1
Natural variantiVAR_027467261F → L in AT3D. 1 Publication1
Natural variantiVAR_007060269E → K in AT3D; type-II; Truro; increases affinity for heparin. 1 PublicationCorresponds to variant dbSNP:rs758087836EnsemblClinVar.1
Natural variantiVAR_007061273 – 307Missing in AT3D; type-I. 1 PublicationAdd BLAST35
Natural variantiVAR_007062283M → I in AT3D; type-II. 2 Publications1
Natural variantiVAR_027468283M → V in AT3D; type-II. 1 Publication1
Natural variantiVAR_071204293R → P in AT3D. 1 Publication1
Natural variantiVAR_007063302L → P in AT3D; type-I. 1
Natural variantiVAR_007064316I → N in AT3D; type-II; Haslar/Whitechapel. 1 Publication1
Natural variantiVAR_027469323S → P in AT3D. 1
Natural variantiVAR_007065334E → K in AT3D; type-II. 1 Publication1
Natural variantiVAR_007066344Missing in AT3D; type-I. 1
Natural variantiVAR_007067381S → P in AT3D; type-I. Corresponds to variant dbSNP:rs121909565EnsemblClinVar.1
Natural variantiVAR_027470397S → P in AT3D; type-I. 1 Publication1
Natural variantiVAR_027471398D → H in AT3D; type-I. 1 Publication1
Natural variantiVAR_071205401H → R in AT3D. 1 Publication1
Natural variantiVAR_027472412S → R in AT3D; type-I. 2 Publications1
Natural variantiVAR_007069414A → T in AT3D; type-II; Hamilton/Glasgow-2; reduces interaction with thrombin by 90%. 4 PublicationsCorresponds to variant dbSNP:rs121909557EnsemblClinVar.1
Natural variantiVAR_007070416A → P in AT3D; type-II; Charleville/Sudbury/Vicenza/Cambridge-1. 1 PublicationCorresponds to variant dbSNP:rs121909548EnsemblClinVar.1
Natural variantiVAR_007071416A → S in AT3D; type-II; Cambridge-2. 2 PublicationsCorresponds to variant dbSNP:rs121909548EnsemblClinVar.1
Natural variantiVAR_007072419A → V in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs121909568EnsemblClinVar.1
Natural variantiVAR_007073424G → D in AT3D; type-II; Stockholm. 2 PublicationsCorresponds to variant dbSNP:rs121909566EnsemblClinVar.1
Natural variantiVAR_007075425R → C in AT3D; type-II. 5 PublicationsCorresponds to variant dbSNP:rs121909554EnsemblClinVar.1
Natural variantiVAR_007074425R → H in AT3D; type-II; Glasgow/Sheffield/Chicago/Avranches/Kumamoto-2; increases affinity for heparin; deprived of inhibitory activity. 5 PublicationsCorresponds to variant dbSNP:rs121909549EnsemblClinVar.1
Natural variantiVAR_007076425R → P in AT3D; type-II; Pescara; deprived of inhibitory of activity. 1 PublicationCorresponds to variant dbSNP:rs121909549EnsemblClinVar.1
Natural variantiVAR_007077426S → L in AT3D; type-II; Denver/Milano-2; deprived of inhibitory activity. 3 PublicationsCorresponds to variant dbSNP:rs121909550EnsemblClinVar.1
Natural variantiVAR_007078434F → C in AT3D; type-II; Rosny. 1 Publication1
Natural variantiVAR_007080434F → L in AT3D; type-II; Maisons-Laffite. 1 Publication1
Natural variantiVAR_007079434F → S in AT3D; type-II; Torino. 1 Publication1
Natural variantiVAR_007081436A → T in AT3D; type-II; Oslo/Paris-3. 1 PublicationCorresponds to variant dbSNP:rs121909546EnsemblClinVar.1
Natural variantiVAR_007082437N → K in AT3D; type-II; La Rochelle. 1 PublicationCorresponds to variant dbSNP:rs1301351856Ensembl.1
Natural variantiVAR_009258438R → G in AT3D; type I and type-II. 2 Publications1
Natural variantiVAR_007083438R → M in AT3D; type-II; Kyoto. 2 Publications1
Natural variantiVAR_071206439P → A in AT3D. 1 Publication1
Natural variantiVAR_007084439P → L in AT3D; type-II; Utah; deprived of inhibitory activity. 2 PublicationsCorresponds to variant dbSNP:rs121909555EnsemblClinVar.1
Natural variantiVAR_007085439P → T in AT3D; type-II; Budapest-5. 1 PublicationCorresponds to variant dbSNP:rs1487411568Ensembl.1
Natural variantiVAR_027473441L → P in AT3D; type-II. 1 PublicationCorresponds to variant dbSNP:rs1188571702Ensembl.1
Natural variantiVAR_007086453I → T in AT3D; type-I. 2 Publications1
Natural variantiVAR_007087456G → R in AT3D; type-I. 2 Publications1
Natural variantiVAR_007088457R → T in AT3D; type-II. 1 Publication1
Natural variantiVAR_007089459 – 461Missing in AT3D; type-I. 3
Natural variantiVAR_007090459A → D in AT3D; type-I. 1 Publication1
Natural variantiVAR_007091461P → L in AT3D; type-II; Budapest. 1 PublicationCorresponds to variant dbSNP:rs121909564EnsemblClinVar.1
Natural variantiVAR_007092462C → F in AT3D; type-I. 2 Publications1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi414A → K: Reduces interaction with thrombin by 99%. 1 Publication1
Mutagenesisi414A → Q: Reduces interaction with thrombin by 80%. 1 Publication1

Keywords - Diseasei

Disease mutation, Thrombophilia

Organism-specific databases

DisGeNET

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DisGeNETi
462

MalaCards human disease database

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MalaCardsi
SERPINC1
MIMi613118 phenotype

Open Targets

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OpenTargetsi
ENSG00000117601

Orphanet; a database dedicated to information on rare diseases and orphan drugs

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Orphaneti
82 Hereditary thrombophilia due to congenital antithrombin deficiency

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA35026

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...
Pharosi
P01008

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...
ChEMBLi
CHEMBL1950

Drug and drug target database

More...
DrugBanki
DB11598 Antithrombin III human
DB00407 Ardeparin
DB09258 Bemiparin
DB09130 Copper
DB06779 Dalteparin
DB06754 Danaparoid
DB01225 Enoxaparin
DB00569 Fondaparinux
DB01109 Heparin
DB04464 N-Formylmethionine
DB08813 Nadroparin
DB09141 Protamine sulfate
DB05361 SR-123781A
DB06271 Sulodexide
DB06822 Tinzaparin

DrugCentral

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DrugCentrali
P01008

IUPHAR/BPS Guide to PHARMACOLOGY

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GuidetoPHARMACOLOGYi
2632

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
SERPINC1

Domain mapping of disease mutations (DMDM)

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DMDMi
113936

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section denotes the presence of an N-terminal signal peptide.<p><a href='/help/signal' target='_top'>More...</a></p>Signal peptidei1 – 321 PublicationAdd BLAST32
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000003248933 – 464Antithrombin-IIIAdd BLAST432

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the PTM / Processing":/help/ptm_processing_section section describes the positions of cysteine residues participating in disulfide bonds.<p><a href='/help/disulfid' target='_top'>More...</a></p>Disulfide bondi40 ↔ 1601 Publication
Disulfide bondi53 ↔ 1271 Publication
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei63Phosphothreonine; by FAM20C1 Publication1
Modified residuei68Phosphoserine; by FAM20CCombined sources1 Publication1
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section specifies the position and type of each covalently attached glycan group (mono-, di-, or polysaccharide).<p><a href='/help/carbohyd' target='_top'>More...</a></p>Glycosylationi128N-linked (GlcNAc...) asparagine4 Publications1
Glycosylationi167N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi187N-linked (GlcNAc...) (complex) asparagine6 Publications1
Glycosylationi224N-linked (GlcNAc...) asparagine3 Publications1
Disulfide bondi279 ↔ 4621 Publication

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

Phosphorylated by FAM20C in the extracellular medium.1 Publication

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

The CPTAC Assay portal

More...
CPTACi
non-CPTAC-1070
non-CPTAC-1071
non-CPTAC-1072

Encyclopedia of Proteome Dynamics

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EPDi
P01008

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
P01008

MassIVE - Mass Spectrometry Interactive Virtual Environment

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MassIVEi
P01008

MaxQB - The MaxQuant DataBase

More...
MaxQBi
P01008

PaxDb, a database of protein abundance averages across all three domains of life

More...
PaxDbi
P01008

PeptideAtlas

More...
PeptideAtlasi
P01008

PRoteomics IDEntifications database

More...
PRIDEi
P01008

ProteomicsDB: a multi-organism proteome resource

More...
ProteomicsDBi
12609
51299

2D gel databases

DOSAC-COBS 2D-PAGE database

More...
DOSAC-COBS-2DPAGEi
P01008

REPRODUCTION-2DPAGE

More...
REPRODUCTION-2DPAGEi
P01008

Two-dimensional polyacrylamide gel electrophoresis database from the Geneva University Hospital

More...
SWISS-2DPAGEi
P01008

PTM databases

CarbonylDB database of protein carbonylation sites

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CarbonylDBi
P01008

GlyConnect protein glycosylation platform

More...
GlyConnecti
766

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
P01008

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
P01008

UniCarbKB; an annotated and curated database of glycan structures

More...
UniCarbKBi
P01008

Miscellaneous databases

CutDB - Proteolytic event database

More...
PMAP-CutDBi
P01008

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Found in plasma.

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000117601 Expressed in 84 organ(s), highest expression level in right lobe of liver

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P01008 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P01008 HS

Organism-specific databases

Human Protein Atlas

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HPAi
CAB016790
HPA001816
HPA024007

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Forms protease inhibiting heterodimer with TMPRSS7.

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
106953, 16 interactors

Database of interacting proteins

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DIPi
DIP-38009N

Protein interaction database and analysis system

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IntActi
P01008, 13 interactors

STRING: functional protein association networks

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STRINGi
9606.ENSP00000356671

Chemistry databases

BindingDB database of measured binding affinities

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BindingDBi
P01008

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1464
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
P01008

Database of comparative protein structure models

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ModBasei
Search...

Protein Data Bank in Europe - Knowledge Base

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PDBe-KBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

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EvolutionaryTracei
P01008

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the serpin family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG2392 Eukaryota
COG4826 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000157967

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P01008

KEGG Orthology (KO)

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KOi
K03911

Identification of Orthologs from Complete Genome Data

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OMAi
DITMVII

Database of Orthologous Groups

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OrthoDBi
165238at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
P01008

TreeFam database of animal gene trees

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TreeFami
TF343094

Family and domain databases

Conserved Domains Database

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CDDi
cd02045 antithrombin-III_like, 1 hit

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
2.30.39.10, 1 hit
3.30.497.10, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR033829 Antithrombin_domain
IPR015555 AT-III
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
IPR042178 Serpin_sf_1
IPR042185 Serpin_sf_2

The PANTHER Classification System

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PANTHERi
PTHR11461 PTHR11461, 1 hit
PTHR11461:SF53 PTHR11461:SF53, 1 hit

Pfam protein domain database

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Pfami
View protein in Pfam
PF00079 Serpin, 1 hit

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00093 SERPIN, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF56574 SSF56574, 1 hit

PROSITE; a protein domain and family database

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PROSITEi
View protein in PROSITE
PS00284 SERPIN, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

P01008-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MYSNVIGTVT SGKRKVYLLS LLLIGFWDCV TCHGSPVDIC TAKPRDIPMN
60 70 80 90 100
PMCIYRSPEK KATEDEGSEQ KIPEATNRRV WELSKANSRF ATTFYQHLAD
110 120 130 140 150
SKNDNDNIFL SPLSISTAFA MTKLGACNDT LQQLMEVFKF DTISEKTSDQ
160 170 180 190 200
IHFFFAKLNC RLYRKANKSS KLVSANRLFG DKSLTFNETY QDISELVYGA
210 220 230 240 250
KLQPLDFKEN AEQSRAAINK WVSNKTEGRI TDVIPSEAIN ELTVLVLVNT
260 270 280 290 300
IYFKGLWKSK FSPENTRKEL FYKADGESCS ASMMYQEGKF RYRRVAEGTQ
310 320 330 340 350
VLELPFKGDD ITMVLILPKP EKSLAKVEKE LTPEVLQEWL DELEEMMLVV
360 370 380 390 400
HMPRFRIEDG FSLKEQLQDM GLVDLFSPEK SKLPGIVAEG RDDLYVSDAF
410 420 430 440 450
HKAFLEVNEE GSEAAASTAV VIAGRSLNPN RVTFKANRPF LVFIREVPLN
460
TIIFMGRVAN PCVK
Length:464
Mass (Da):52,602
Last modified:July 21, 1986 - v1
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i9A4E324F00683D9D
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q8TCE1Q8TCE1_HUMAN
Antithrombin-III
SERPINC1
259Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti69 – 70EQ → QE AA sequence (Ref. 10) Curated2
Sequence conflicti77N → NN in BAA06212 (Ref. 3) Curated1
Sequence conflicti97H → R in AAG35525 (Ref. 5) Curated1
Sequence conflicti120A → T in BAG35537 (PubMed:14702039).Curated1
Sequence conflicti226T → A in BAG35537 (PubMed:14702039).Curated1
Sequence conflicti247 – 249Missing AA sequence (Ref. 10) Curated3
Sequence conflicti388Missing AA sequence (PubMed:7734359).Curated1
Sequence conflicti395Y → YA AA sequence (PubMed:7734359).Curated1

<p>This subsection of the ‘Sequence’ section reports information derived from mass spectrometry experiments done on the entire protein or on biologically active derived peptide(s).<p><a href='/help/mass_spectrometry' target='_top'>More...</a></p>Mass spectrometryi

Molecular mass is 57863 Da from positions 33 - 464. Determined by ESI. 1 Publication
Molecular mass is 57911 Da from positions 33 - 464. Determined by ESI. Variant Thr-414.1 Publication

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02745017Y → S in AT3D; type-I. 1 Publication1
Natural variantiVAR_01274823L → P in AT3D; type-I; does not undergo post-translational glycosylation. 2 PublicationsCorresponds to variant dbSNP:rs387906575EnsemblClinVar.1
Natural variantiVAR_00703230V → E in Dublin. 4 PublicationsCorresponds to variant dbSNP:rs2227624EnsemblClinVar.1
Natural variantiVAR_02745132C → R in AT3D; type-I. 1 Publication1
Natural variantiVAR_00703339I → N in AT3D; type-II; Rouen-3; lack of heparin-binding properties. 1 PublicationCorresponds to variant dbSNP:rs121909558EnsemblClinVar.1
Natural variantiVAR_00703452M → T Previously Whitechapel. 1 PublicationCorresponds to variant dbSNP:rs892712171Ensembl.1
Natural variantiVAR_07119953C → F in AT3D. 1 Publication1
Natural variantiVAR_00703556R → C in AT3D; type-II; Rouen-4; lack of heparin-binding properties. 2 PublicationsCorresponds to variant dbSNP:rs28929469EnsemblClinVar.1
Natural variantiVAR_00703673P → L in AT3D; type-II; lacks heparin-binding ability. 4 PublicationsCorresponds to variant dbSNP:rs121909551EnsemblClinVar.1
Natural variantiVAR_00703779R → C in AT3D; Tours/Alger/Amiens/Toyama/Paris-1/Paris-2/Padua-2/Barcelona-2/Kumamoto/Omura/Sasebo; lacks heparin-binding ability. 3 PublicationsCorresponds to variant dbSNP:rs121909547EnsemblClinVar.1
Natural variantiVAR_00703879R → H in AT3D; type-II; Rouen-1/Padua-1/Bligny/Budapest-2; lack of heparin-binding properties. 2 PublicationsCorresponds to variant dbSNP:rs121909552EnsemblClinVar.1
Natural variantiVAR_00703979R → S in AT3D; type-II; Rouen-2; lack of heparin-binding properties. 1 PublicationCorresponds to variant dbSNP:rs121909547EnsemblClinVar.1
Natural variantiVAR_00704087Missing in AT3D; type-I. 1 Publication1
Natural variantiVAR_00704189R → C in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs147266200Ensembl.1
Natural variantiVAR_00704290F → L in AT3D; type-I; Budapest-6. 1 Publication1
Natural variantiVAR_02745295Y → C in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs907768931Ensembl.1
Natural variantiVAR_01231695Y → S in AT3D; type-I. 2 Publications1
Natural variantiVAR_02745398L → P in AT3D; type-I. 1 Publication1
Natural variantiVAR_007043108 – 109Missing in AT3D; type-I. 2
Natural variantiVAR_007044112P → T in AT3D; type-I. 2 Publications1
Natural variantiVAR_027454121M → K in AT3D; type-I. 1 Publication1
Natural variantiVAR_071200125G → D in AT3D. 1 Publication1
Natural variantiVAR_027455127C → R in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs121909573EnsemblClinVar.1
Natural variantiVAR_007045131L → F in AT3D; type-II; Budapest-3/Budapest-7. 2 PublicationsCorresponds to variant dbSNP:rs121909567EnsemblClinVar.1
Natural variantiVAR_007046131L → V in AT3D; type-II; Southport. 2 Publications1
Natural variantiVAR_007047133Q → K in AT3D; type I. 1 PublicationCorresponds to variant dbSNP:rs1411331203Ensembl.1
Natural variantiVAR_007048138 – 139Missing in AT3D; type-I. 1 Publication2
Natural variantiVAR_027456146K → E in AT3D; Dreux; complete loss af heparin binding. 1 PublicationCorresponds to variant dbSNP:rs1170430756Ensembl.1
Natural variantiVAR_013085147T → A1 PublicationCorresponds to variant dbSNP:rs2227606Ensembl.1
Natural variantiVAR_007049148S → P in AT3D; type-II; Nagasaki; defective heparin binding associated with thrombosis. 2 PublicationsCorresponds to variant dbSNP:rs121909569EnsemblClinVar.1
Natural variantiVAR_007050150Q → P in AT3D; type-II; Vienna. 2 PublicationsCorresponds to variant dbSNP:rs765445413Ensembl.1
Natural variantiVAR_012749152 – 154Missing in AT3D; type-I. 1 Publication3
Natural variantiVAR_007051152H → Y in AT3D; type-I. 1 Publication1
Natural variantiVAR_007052153Missing in AT3D; type-I. 1
Natural variantiVAR_007053158L → P in AT3D; type-I. 1 Publication1
Natural variantiVAR_027457160C → Y in AT3D; type-I. 2 Publications1
Natural variantiVAR_007054161R → Q in AT3D; type-II; Geneva. 2 PublicationsCorresponds to variant dbSNP:rs121909563EnsemblClinVar.1
Natural variantiVAR_012750167N → T1 PublicationCorresponds to variant dbSNP:rs121909570EnsemblClinVar.1
Natural variantiVAR_071201170S → P in AT3D. 1 Publication1
Natural variantiVAR_027458178L → H in AT3D; type-I. 1 Publication1
Natural variantiVAR_027459179F → L in AT3D; type-I. 1 Publication1
Natural variantiVAR_007055190Y → C Polymorphism in population of Scandinavian origin. 2 Publications1
Natural variantiVAR_007056198Y → C in AT3D; type-I and -II; Whitechapel. 1 PublicationCorresponds to variant dbSNP:rs1425532034Ensembl.1
Natural variantiVAR_027460198Y → H in AT3D; type-I. 2 Publications1
Natural variantiVAR_027461214S → F in AT3D; type-I. 1 Publication1
Natural variantiVAR_007057214S → Y in AT3D; type-I. Corresponds to variant dbSNP:rs483352854EnsemblClinVar.1
Natural variantiVAR_071202218I → N in AT3D. 1 Publication1
Natural variantiVAR_027462218Missing in AT3D; type-I. 1 Publication1
Natural variantiVAR_007059219N → D in AT3D; type-II; Rouen-6; increases affinity for heparin. 2 PublicationsCorresponds to variant dbSNP:rs121909571EnsemblClinVar.1
Natural variantiVAR_007058219N → K in AT3D; type-II; Glasgow-3. 1 Publication1
Natural variantiVAR_027463223S → P in AT3D; type-I. 2 PublicationsCorresponds to variant dbSNP:rs121909572EnsemblClinVar.1
Natural variantiVAR_027464243T → I in AT3D; type-I. 1 Publication1
Natural variantiVAR_071203248V → G in AT3D. 1 Publication1
Natural variantiVAR_027465251I → T in AT3D; type-I. 1 PublicationCorresponds to variant dbSNP:rs1423630663Ensembl.1
Natural variantiVAR_027466257W → R in AT3D; type-I. 2 Publications1
Natural variantiVAR_027467261F → L in AT3D. 1 Publication1
Natural variantiVAR_007060269E → K in AT3D; type-II; Truro; increases affinity for heparin. 1 PublicationCorresponds to variant dbSNP:rs758087836EnsemblClinVar.1
Natural variantiVAR_007061273 – 307Missing in AT3D; type-I. 1 PublicationAdd BLAST35
Natural variantiVAR_007062283M → I in AT3D; type-II. 2 Publications1
Natural variantiVAR_027468283M → V in AT3D; type-II. 1 Publication1
Natural variantiVAR_071204293R → P in AT3D. 1 Publication1
Natural variantiVAR_007063302L → P in AT3D; type-I. 1
Natural variantiVAR_007064316I → N in AT3D; type-II; Haslar/Whitechapel. 1 Publication1
Natural variantiVAR_027469323S → P in AT3D. 1
Natural variantiVAR_007065334E → K in AT3D; type-II. 1 Publication1
Natural variantiVAR_007066344Missing in AT3D; type-I. 1
Natural variantiVAR_007067381S → P in AT3D; type-I. Corresponds to variant dbSNP:rs121909565EnsemblClinVar.1
Natural variantiVAR_007068391R → Q. Corresponds to variant dbSNP:rs201541724Ensembl.1
Natural variantiVAR_027470397S → P in AT3D; type-I. 1 Publication1
Natural variantiVAR_027471398D → H in AT3D; type-I. 1 Publication1
Natural variantiVAR_071205401H → R in AT3D. 1 Publication1
Natural variantiVAR_027472412S → R in AT3D; type-I. 2 Publications1
Natural variantiVAR_007069414A → T in AT3D; type-II; Hamilton/Glasgow-2; reduces interaction with thrombin by 90%. 4 PublicationsCorresponds to variant dbSNP:rs121909557EnsemblClinVar.1
Natural variantiVAR_007070416A → P in AT3D; type-II; Charleville/Sudbury/Vicenza/Cambridge-1. 1 PublicationCorresponds to variant dbSNP:rs121909548EnsemblClinVar.1
Natural variantiVAR_007071416A → S in AT3D; type-II; Cambridge-2. 2 PublicationsCorresponds to variant dbSNP:rs121909548EnsemblClinVar.