Antithrombin-III

Function

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.

Classification

Family (Pfam)

PF00079 Serpin

InterPro

Antithrombin_3_serpin_domain, Serpin_CS, Serpin_dom, Serpin_fam, Serpin_sf, Serpin_sf_1, Serpin_sf_2

Functional cluster

Cytochrome P450 & Heme Enzymes

Experimental structures · PDB · 27

• 1ANT X-ray 3.00A
• 1ATH X-ray 3.20A
• 1AZX X-ray 2.90A
• 1BR8 X-ray 2.90A
• 1DZG X-ray 2.80A
• 1DZH X-ray 2.85A
• 1E03 X-ray 2.90A
• 1E04 X-ray 2.60A
• 1E05 X-ray 2.62A
• 1JVQ X-ray 2.60A
• 1LK6 X-ray 2.80A
• 1NQ9 X-ray 2.60A
• … and 15 more

A predicted model is available from AlphaFold.

Gene Ontology · 13

• GO:0072562 blood microparticle
• GO:0005788 endoplasmic reticulum lumen
• GO:0070062 extracellular exosome
• GO:0031012 extracellular matrix
• GO:0005576 extracellular region
• GO:0005615 extracellular space
• GO:0005886 plasma membrane
• GO:0008201 heparin binding
• GO:0042802 identical protein binding
• GO:0002020 protease binding
• GO:0004867 serine-type endopeptidase inhibitor activity
• GO:0007596 blood coagulation
• GO:0030193 regulation of blood coagulation

Disease associations

• hereditary antithrombin deficiency MONDO:0013144

Drugs targeting this protein · 1

• FONDAPARINUX SODIUM activator

Related proteins · sequence + function similarity

• Antithrombin-III 1.00
• Antithrombin-III 0.98
• Antithrombin-III 0.98
• Antithrombin-III 0.98
• Intracellular coagulation inhibitor 2 0.93
• Serpin B11 0.91
• Serpin B10 0.91
• Intracellular coagulation inhibitor 3 0.90
• Iripin-1 0.90
• Serpin B7 0.90
• Intracellular coagulation inhibitor 1 0.90
• Iripin-2 0.90

Co-cited proteins · studied together in the literature

• Plasma protease C1 inhibitor 1 shared papers
• Transmembrane protease serine 7 1 shared papers
• Transmembrane protease serine 7 1 shared papers
• Alpha-2-antiplasmin 1 shared papers
• Plasminogen activator inhibitor 1 1 shared papers
• Plasma serine protease inhibitor 1 shared papers
• Beta-2-glycoprotein 1 1 shared papers
• IgGFc-binding protein 1 shared papers
• Aminopeptidase N 1 shared papers
• Zinc-alpha-2-glycoprotein 1 shared papers
• Macrosialin 1 shared papers
• Sushi domain-containing protein 1 1 shared papers

Literature · 70 cited papers

• Antithrombin p.Thr147Ala: The first founder mutation in people of African origin responsible for inherited antithrombin deficiency. Thromb. Haemost. · 2021
• Defects of splicing in antithrombin deficiency. Res. Pract. Thromb. Haemost. · 2017
• A single kinase generates the majority of the secreted phosphoproteome. Cell · 2015
• An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
• Type II antithrombin deficiency caused by a founder mutation Pro73Leu in the Finnish population: clinical picture. J. Thromb. Haemost. · 2013
• Type II antithrombin deficiency caused by a large in-frame insertion: structural, functional and pathological relevance. J. Thromb. Haemost. · 2012
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• Enrichment of glycopeptides for glycan structure and attachment site identification. Nat. Methods · 2009
• Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry. J. Proteome Res. · 2009
• Retinal vein occlusion associated with antithrombin deficiency secondary to a novel G9840C missense mutation. Arch. Ophthalmol. · 2006
• The DNA sequence and biological annotation of human chromosome 1. Nature · 2006
• Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry. J. Proteome Res. · 2005
• … and 58 more in the literature graph