Coagulation factor XI

Function

Factor XI triggers the middle phase of the intrinsic pathway of blood coagulation by activating factor IX.

Classification

Family (Pfam)

PF00024 PAN_1, PF00089 Trypsin

InterPro

Apple, Pan_app, Peptidase_S1_PA, Peptidase_S1_PA_chymotrypsin, Peptidase_S1A, Trypsin_dom, TRYPSIN_HIS, TRYPSIN_SER

Functional cluster

Venom Serine Proteases & Phospholipase A2

Experimental structures · PDB · 114

• 1XX9 X-ray 2.20A
• 1XXD X-ray 2.91A
• 1XXF X-ray 2.60A
• 1ZHM X-ray 1.96A
• 1ZHP X-ray 2.70A
• 1ZHR X-ray 1.73A
• 1ZJD X-ray 2.60A
• 1ZLR X-ray 2.50A
• 1ZMJ X-ray 2.00A
• 1ZML X-ray 2.25A
• 1ZMN X-ray 2.05A
• 1ZOM X-ray 2.25A
• … and 102 more

A predicted model is available from AlphaFold.

Gene Ontology · 13

• GO:0070062 extracellular exosome
• GO:0005576 extracellular region
• GO:0005615 extracellular space
• GO:0016020 membrane
• GO:0005886 plasma membrane
• GO:0008201 heparin binding
• GO:0042802 identical protein binding
• GO:0070009 serine-type aminopeptidase activity
• GO:0004252 serine-type endopeptidase activity
• GO:0008236 serine-type peptidase activity
• GO:0007596 blood coagulation
• GO:0031639 plasminogen activation
• GO:0051919 positive regulation of fibrinolysis

Disease associations

• congenital factor XI deficiency MONDO:0012897

Drugs targeting this protein · 1

• MILVEXIAN inhibitor

Related proteins · sequence + function similarity

• Coagulation factor XI 0.98
• Coagulation factor XI 0.98
• Plasma kallikrein 0.97
• Plasma kallikrein 0.97
• Plasma kallikrein 0.96
• Plasma kallikrein 0.96
• Salivary plasminogen activator alpha 1 0.78
• Tissue-type plasminogen activator 0.78
• Salivary plasminogen activator alpha 2 0.78
• Transmembrane protease serine 7 0.78
• Transmembrane protease serine 7 0.78
• Tissue-type plasminogen activator 0.78

Co-cited proteins · studied together in the literature

• Coagulation factor XII 3 shared papers
• Prothrombin 2 shared papers
• Coagulation factor IX 1 shared papers
• Long form salivary protein D7L1 1 shared papers
• Long form salivary protein D7L2 1 shared papers
• Plasma kallikrein 1 shared papers
• Guianensin 1 shared papers
• Coagulation factor X 1 shared papers
• Long form salivary protein D7L3 1 shared papers
• Coagulation factor X 1 shared papers
• Coagulation factor V 1 shared papers
• Plasma serine protease inhibitor 1 shared papers

Literature · 41 cited papers

• Guianensin, a Simulium guianense salivary protein, has broad anti-hemostatic and anti-inflammatory properties. Front. Immunol. · 2023
• Novel salivary antihemostatic activities of long-form D7 proteins from the malaria vector Anopheles gambiae facilitate hematophagy. J. Biol. Chem. · 2022
• Molecular basis and bleeding manifestations of factor XI deficiency in 11 Turkish families. Blood Coagul. Fibrinolysis · 2015
• Site-specific N-glycosylation analysis of human factor XI: Identification of a noncanonical NXC glycosite. Proteomics · 2014
• A sequential mechanism for exosite-mediated factor IX activation by factor XIa. J. Biol. Chem. · 2012
• Identification of a novel F11 missense mutation (Ile463Ser) in a family with congenital factor XI deficiency. Blood Coagul. Fibrinolysis · 2012
• Revisiting the molecular epidemiology of factor XI deficiency: nine new mutations and an original large 4qTer deletion in western Brittany (France). Thromb. Haemost. · 2012
• A novel missense mutation Asp506Gly in exon 13 of the F11 gene in an asymptomatic Korean woman with mild factor XI deficiency. Korean J. Lab. Med. · 2011
• A cluster of factor XI-deficient patients due to a new mutation (Ile 436 Lys) in northeastern Italy. Eur. J. Haematol. · 2012
• Polyphosphate is a cofactor for the activation of factor XI by thrombin. Blood · 2011
• Population-specific spectrum of the F11 mutations in Koreans: evidence for a founder effect. Clin. Genet. · 2012
• Three dominant-negative mutations in factor XI-deficient patients. Haemophilia · 2011
• … and 29 more in the literature graph