Coagulation Factor XI (F11)

FXI; PTA; Fletcher Factor; Prekallikrein; Plasma Thromboplastin Antecedent

SPN	V007
HGNC	3529
MGI	99481
PubMed	10961575
RGD	1309364
UniProt	P03951
Wiki	Factor_XI

Factor XI is the zymogen form of factor XIa, one of the enzymes of the coagulation cascade. Like many other coagulation factors, it is a serine protease.Factor XI (FXI) is produced by the liver and circulates as a homo-dimer in its inactive form. The plasma half-life of FXI is approximately 52 hours.

The zymogen factor is activated into factor XIa by factor XIIa (FXIIa), thrombin, and it is also autocatalytic, and FXI is a member of the "contact pathway" due to activation by FXIIa (with includes HMWK, prekallikrein, factor XII, factor XI and factor IX).Factor XIa activates factor IX by selectively cleaving arg-ala and arg-val peptide bonds. Factor IXa, in turn, activates factor X.Inhibitors of factor XIa include protein Z-dependent protease inhibitor (ZPI), which is independent of protein Z.

Reference

"Amino acid sequence of human factor XI, a blood coagulation factor with four tandem repeats that are highly homologous with plasma prekallikrein."
Biochemistry 25:2417-2424(1986) [PubMed] [Europe PMC] [Abstract]

"Organization of the gene for human factor XI."
Biochemistry 26:7221-7228(1987) [PubMed] [Europe PMC] [Abstract]

"Molecular cloning of platelet factor XI, an alternative splicing product of the plasma factor XI gene."
J. Biol. Chem. 273:13787-13793(1998) [PubMed] [Europe PMC] [Abstract]

"Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Location of the disulfide bonds in human coagulation factor XI: the presence of tandem apple domains."
Biochemistry 30:2056-2060(1991) [PubMed] [Europe PMC] [Abstract]

"Inactivation of human plasma kallikrein and factor XIa by protein C inhibitor."
Biochemistry 27:4231-4237(1988) [PubMed] [Europe PMC] [Abstract]

"Localization of a heparin binding site in the catalytic domain of factor XIa."
Biochemistry 40:7569-7580(2001) [PubMed] [Europe PMC] [Abstract]

"Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]

"Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]

"Site-specific N-glycosylation analysis of human factor XI: Identification of a noncanonical NXC glycosite."
Proteomics 14:2460-2470(2014) [PubMed] [Europe PMC] [Abstract]

"Clavatadine A, a natural product with selective recognition and irreversible inhibition of factor XIa."
J. Med. Chem. 51:3583-3587(2008) [PubMed] [Europe PMC] [Abstract]

"Factor XI (plasma thromboplastin antecedent) deficiency in Ashkenazi Jews is a bleeding disorder that can result from three types of point mutations."
Proc. Natl. Acad. Sci. U.S.A. 86:7667-7671(1989) [PubMed] [Europe PMC] [Abstract]

"Expression of human blood coagulation factor XI: characterization of the defect in factor XI type III deficiency."
Blood 79:1435-1440(1992) [PubMed] [Europe PMC] [Abstract]

"Six point mutations that cause factor XI deficiency."
Blood 85:1509-1516(1995) [PubMed] [Europe PMC] [Abstract]

"Identification of two novel mutations in non-Jewish factor XI deficiency."
Br. J. Haematol. 90:916-920(1995) [PubMed] [Europe PMC] [Abstract]

"Severe factor XI deficiency in an Arab family associated with a novel mutation in exon 11."
Br. J. Haematol. 99:575-577(1997) [PubMed] [Europe PMC] [Abstract]

"Identification of mutations and polymorphisms in the factor XI genes of an African American family by dideoxyfingerprinting."
Blood 92:3309-3317(1998) [PubMed] [Europe PMC] [Abstract]

Erratum
Blood 93:1786-1786(1999)

"Identification of a novel mutation in a non-Jewish factor XI deficient kindred."
Br. J. Haematol. 104:44-49(1999) [PubMed] [Europe PMC] [Abstract]

"Heterozygous factor XI deficiency associated with three novel mutations."
Br. J. Haematol. 107:763-765(1999) [PubMed] [Europe PMC] [Abstract]

"Characterization of single-nucleotide polymorphisms in coding regions of human genes."
Nat. Genet. 22:231-238(1999) [PubMed] [Europe PMC] [Abstract]

Erratum
Nat. Genet. 23:373-373(1999)

"Factor XI deficiency in French Basques is caused predominantly by an ancestral Cys38Arg mutation in the factor XI gene."
Blood 99:2448-2454(2002) [PubMed] [Europe PMC] [Abstract]

"Dominant factor XI deficiency caused by mutations in the factor XI catalytic domain."
Blood 104:128-134(2004) [PubMed] [Europe PMC] [Abstract]

"Severe factor XI deficiency caused by compound heterozygosity."
Br. J. Haematol. 125:817-818(2004) [PubMed] [Europe PMC] [Abstract]

"Genetic analysis in FXI deficiency: six novel mutations and the use of a polymerase chain reaction-based test to define a whole gene deletion."
Br. J. Haematol. 129:825-829(2005) [PubMed] [Europe PMC] [Abstract]

"Identification of five novel mutations in the factor XI gene (F11) of patients with factor XI deficiency."
Blood Coagul. Fibrinolysis 17:69-73(2006) [PubMed] [Europe PMC] [Abstract]

"Seven novel point mutations in the F11 gene in Iranian FXI-deficient patients."
Haemophilia 14:91-95(2008) [PubMed] [Europe PMC] [Abstract]

"Population-specific spectrum of the F11 mutations in Koreans: evidence for a founder effect."
Clin. Genet. 82:180-186(2012) [PubMed] [Europe PMC] [Abstract]

"Three dominant-negative mutations in factor XI-deficient patients."
Haemophilia 17:E919-E922(2011) [PubMed] [Europe PMC] [Abstract]

"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. 31:290-293(2011) [PubMed] [Europe PMC] [Abstract]

"Identification of a novel F11 missense mutation (Ile463Ser) in a family with congenital factor XI deficiency."
Blood Coagul. Fibrinolysis 23:251-252(2012) [PubMed] [Europe PMC] [Abstract]

"A cluster of factor XI-deficient patients due to a new mutation (Ile 436 Lys) in northeastern Italy."
Eur. J. Haematol. 88:229-236(2012) [PubMed] [Europe PMC] [Abstract]

"Revisiting the molecular epidemiology of factor XI deficiency: nine new mutations and an original large 4qTer deletion in western Brittany (France)."
Thromb. Haemost. 107:44-50(2012) [PubMed] [Europe PMC] [Abstract]