factor XI

同

plasma thromboplastin antecedent

WordNet

1. be a contributing factor; "make things factor into a companys profitability"
2. any of the numbers (or symbols) that form a product when multiplied together
3. an independent variable in statistics
4. anything that contributes causally to a result; "a number of factors determined the outcome"
5. consider as relevant when making a decision; "You must factor in the recent developments" (同)factor in, factor out
6. resolve into factors; "a quantum computer can factor the number 15" (同)factor in, factor out
7. an event known to have happened or something known to have existed; "your fears have no basis in fact"; "how much of the story is fact and how much fiction is hard to tell"
8. a concept whose truth can be proved; "scientific hypotheses are not facts"
9. a piece of information about circumstances that exist or events that have occurred; "first you must collect all the facts of the case"
10. a statement or assertion of verified information about something that is the case or has happened; "he supported his argument with an impressive array of facts"
11. the 14th letter of the Greek alphabet
12. the 24th letter of the Roman alphabet (同)x, ex

PrepTutorEJDIC

1. (…の)『要因』,(…を生み出す)要素《+『in』+『名』(do『ing』)》 / 囲数,約数 / 代理人,《おもに英》仲買人 / =factorize
2. 〈C〉『事実』,実際にある(あった)事 / 〈U〉真相,真実(truth) / 《the~》(法律用語で)犯行
3. クシー(ギリシア語アルファベットの第14字Ξ,ξ;英語のX,xに相当)
4. Christ / Christian

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/02/21 23:37:18」(JST)

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Coagulation factor XI
PDB rendering based on 1xx9.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1XX9​, 1XXD​, 1XXF​, 1ZHM​, 1ZHP​, 1ZHR​, 1ZJD​, 1ZLR​, 1ZMJ​, 1ZML​, 1ZMN​, 1ZOM​, 1ZPB​, 1ZPC​, 1ZPZ​, 1ZRK​, 1ZSJ​, 1ZSK​, 1ZSL​, 1ZTJ​, 1ZTK​, 1ZTL​, 2F83​, 2FDA​, 2J8J​, 2J8L​, 3BG8​, 3SOR​, 3SOS​, 4CR5​, 4CR9​, 4CRA​, 4CRB​, 4CRC​, 4CRD​, 4CRE​, 4CRF​, 4CRG​, 4NA7​, 4NA8​, 4TY6​, 4TY7​, 4WXI​, 4X6M​, 4X6N​, 4X6O​, 4X6P​
Identifiers
Symbols	F11 ; FXI
External IDs	OMIM: 264900 MGI: 99481 HomoloGene: 86654 ChEMBL: 2820 GeneCards: F11 Gene
EC number	3.4.21.27
Gene ontology Molecular function • serine-type endopeptidase activity • protein binding • heparin binding Cellular component • extracellular region • extracellular space • plasma membrane • membrane • extracellular exosome Biological process • blood coagulation • blood coagulation, intrinsic pathway • plasminogen activation • positive regulation of fibrinolysis Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	2160	109821
Ensembl	ENSG00000088926	ENSMUSG00000031645
UniProt	P03951	Q91Y47
RefSeq (mRNA)	NM_000128	NM_028066
RefSeq (protein)	NP_000119	NP_082342
Location (UCSC)	Chr 4: 186.27 – 186.29 Mb	Chr 8: 45.24 – 45.26 Mb
PubMed search	[1]	[2]

Factor XI or plasma thromboplastin antecedent 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. In humans, Factor XI is encoded by the F11 gene.

Function

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 FXIa itself; due to its activation by FXIIa, FXI is a member of the "contact pathway" (which 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, a member of the serine protease inhibitor/serpin class of proteins), which is independent of protein Z (its action on factor X, however, is protein Z-dependent, hence its name).

Structure

Although synthesized as a single polypeptide chain, FXI circulates as a homodimer. Every chain has a relative molecular mass of approximately 80000. Typical plasma concentrations of FXI are 5 μg/mL, corresponding to a plasma concentration (of FXI dimers) of approximately 30 nM. The FXI gene is 23kb in length, has 15 exons, and is found on chromosome 4q32-35.

Role in disease

Deficiency of factor XI causes the rare hemophilia C; this mainly occurs in Ashkenazi Jews and is believed to affect approximately 8% of that population. Less commonly, hemophilia C can be found in Jews of Iraqi ancestry and in Israeli Arabs. The condition has been described in other populations at around 1% of cases. It is an autosomal recessive disorder. There is little spontaneous bleeding, but surgical procedures may cause excessive blood loss, and prophylaxis is required.

Low levels of factor XI also occur in many other disease states, including Noonan syndrome.

High levels of factor XI have been implicated in thrombosis, although it is uncertain what determines these levels and how serious the procoagulant state is.

See also

• Contact activation pathway (also known as the intrinsic pathway)
• Tissue factor pathway (also known as the extrinsic pathway)

References

Further reading

• Gailani D, Zivelin A, Sinha D, Walsh PN (2005). "Do platelets synthesize factor XI?". Journal of Thrombosis and Haemostasis : JTH 2 (10): 1709–12. doi:10.1111/j.1538-7836.2004.00935.x. PMID 15456479. 
• Dossenbach-Glaninger A, Hopmeier P (Jun 2005). "Coagulation factor XI: a database of mutations and polymorphisms associated with factor XI deficiency". Blood Coagulation & Fibrinolysis : An International Journal in Haemostasis and Thrombosis 16 (4): 231–8. doi:10.1097/01.mbc.0000169214.62560.a5. PMID 15870541. 
• Seligsohn U (Jul 2007). "Factor XI in haemostasis and thrombosis: past, present and future". Thrombosis and Haemostasis 98 (1): 84–9. doi:10.1160/th07-04-0246. PMID 17597996. 
• Meijers JC, Davie EW, Chung DW (Mar 1992). "Expression of human blood coagulation factor XI: characterization of the defect in factor XI type III deficiency". Blood 79 (6): 1435–40. PMID 1547342. 
• Gailani D, Broze GJ (Aug 1991). "Factor XI activation in a revised model of blood coagulation". Science 253 (5022): 909–12. doi:10.1126/science.1652157. PMID 1652157. 
• Buetow KH, Shiang R, Yang P, Nakamura Y, Lathrop GM, White R, Wasmuth JJ, Wood S, Berdahl LD, Leysens NJ (May 1991). "A detailed multipoint map of human chromosome 4 provides evidence for linkage heterogeneity and position-specific recombination rates". American Journal of Human Genetics 48 (5): 911–25. PMC 1683054. PMID 1673289. 
• Bodfish P, Warne D, Watkins C, Nyberg K, Spurr NK (1992). "Dinucleotide repeat polymorphism in the human coagulation factor XI gene, intron B (F11), detected using the polymerase chain reaction". Nucleic Acids Research 19 (24): 6979. doi:10.1093/nar/19.24.6979-a. PMC 329377. PMID 1762944. 
• Clarkson K, Rosenfeld B, Fair J, Klein A, Bell W (Dec 1991). "Factor XI deficiency acquired by liver transplantation". Annals of Internal Medicine 115 (11): 877–9. doi:10.7326/0003-4819-115-11-877. PMID 1952475. 
• McMullen BA, Fujikawa K, Davie EW (Feb 1991). "Location of the disulfide bonds in human coagulation factor XI: the presence of tandem apple domains". Biochemistry 30 (8): 2056–60. doi:10.1021/bi00222a008. PMID 1998667. 
• Naito K, Fujikawa K (Apr 1991). "Activation of human blood coagulation factor XI independent of factor XII. Factor XI is activated by thrombin and factor XIa in the presence of negatively charged surfaces". The Journal of Biological Chemistry 266 (12): 7353–8. PMID 2019570. 
• Asakai R, Chung DW, Davie EW, Seligsohn U (Jul 1991). "Factor XI deficiency in Ashkenazi Jews in Israel". The New England Journal of Medicine 325 (3): 153–8. doi:10.1056/NEJM199107183250303. PMID 2052060. 
• España F, Berrettini M, Griffin JH (Aug 1989). "Purification and characterization of plasma protein C inhibitor". Thrombosis Research 55 (3): 369–84. doi:10.1016/0049-3848(89)90069-8. PMID 2551064. 
• Asakai R, Chung DW, Ratnoff OD, Davie EW (Oct 1989). "Factor XI (plasma thromboplastin antecedent) deficiency in Ashkenazi Jews is a bleeding disorder that can result from three types of point mutations". Proceedings of the National Academy of Sciences of the United States of America 86 (20): 7667–71. doi:10.1073/pnas.86.20.7667. PMC 298131. PMID 2813350. 
• Asakai R, Davie EW, Chung DW (1988). "Organization of the gene for human factor XI". Biochemistry 26 (23): 7221–8. doi:10.1021/bi00397a004. PMID 2827746. 
• Fujikawa K, Chung DW, Hendrickson LE, Davie EW (May 1986). "Amino acid sequence of human factor XI, a blood coagulation factor with four tandem repeats that are highly homologous with plasma prekallikrein". Biochemistry 25 (9): 2417–24. doi:10.1021/bi00357a018. PMID 3636155. 
• Warn-Cramer BJ, Bajaj SP (1986). "Stoichiometry of binding of high molecular weight kininogen to factor XI/XIa". Biochemical and Biophysical Research Communications 133 (2): 417–22. doi:10.1016/0006-291X(85)90922-2. PMID 3936495. 
• Bouma BN, Vlooswijk RA, Griffin JH (Nov 1983). "Immunologic studies of human coagulation factor XI and its complex with high molecular weight kininogen". Blood 62 (5): 1123–31. PMID 6626744. 
• Tuszynski GP, Bevacqua SJ, Schmaier AH, Colman RW, Walsh PN (Jun 1982). "Factor XI antigen and activity in human platelets". Blood 59 (6): 1148–56. PMID 7044446. 
• Imanaka Y, Lal K, Nishimura T, Bolton-Maggs PH, Tuddenham EG, McVey JH (Aug 1995). "Identification of two novel mutations in non-Jewish factor XI deficiency". British Journal of Haematology 90 (4): 916–20. doi:10.1111/j.1365-2141.1995.tb05215.x. PMID 7669672. 
• Pugh RE, McVey JH, Tuddenham EG, Hancock JF (Mar 1995). "Six point mutations that cause factor XI deficiency". Blood 85 (6): 1509–16. PMID 7888672. 
• Riley PW, Cheng H, Samuel D, Roder H, Walsh PN (Mar 2007). "Dimer dissociation and unfolding mechanism of coagulation factor XI apple 4 domain: spectroscopic and mutational analysis". Journal of Molecular Biology 367 (2): 558–73. doi:10.1016/j.jmb.2006.12.066. PMC 1945241. PMID 17257616. 
• Samuel D, Cheng H, Riley PW, Canutescu AA, Nagaswami C, Weisel JW, Bu Z, Walsh PN, Roder H (Oct 2007). "Solution structure of the A4 domain of factor XI sheds light on the mechanism of zymogen activation". Proceedings of the National Academy of Sciences of the United States of America 104 (40): 15693–8. doi:10.1073/pnas.0703080104. PMC 1987390. PMID 17884987. 

External links

• The MEROPS online database for peptidases and their inhibitors: S01.213

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 第ⅩＩ因子欠乏症 factor xi deficiency
• 2. 止血の概要 overview of hemostasis
• 3. 後天性の凝固因子阻害物質 acquired inhibitors of coagulation
• 4. 直接トロンビン阻害剤および直接Xa因子阻害剤による抗凝固療法 anticoagulation with direct thrombin inhibitors and direct factor xa inhibitors
• 5. 稀な遺伝性凝固異常 rare inherited coagulation disorders

English Journal

• Essential roles for platelets during neutrophil-dependent or lymphocyte-mediated defense against bacterial pathogens.

• Wang Z1, Zhao Q, Zhang D, Sun C, Bao C, Yi M, Xing L, Luo D.
• Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis.Blood Coagul Fibrinolysis.2016 Sep;27(6):667-72. doi: 10.1097/MBC.0000000000000455.
• Emerging evidence from animal models suggests that platelets may participate in a wide variety of processes including the immune response against infection. More than 200 whole blood samples from patients and healthy controls were run in the System XE-5000 analyzer, and plasma fractions were separat
• PMID 26588444

• Management of Haemophilia in Developing Countries: Challenges and Options.

• Ghosh K1, Ghosh K2.
• Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion.Indian J Hematol Blood Transfus.2016 Sep;32(3):347-55. doi: 10.1007/s12288-015-0562-x. Epub 2015 Jun 18.
• There are significant challenges in managing haemophilia patients in developing countries. These challenges are (i) Lack of proper health care infrastructure and human resources suitable for haemophilia care (ii) Competing health care priorities of the government. (iii) Lack of penetrance of medical
• PMID 27429529

• ERBB3-rs2292239 as primary type 1 diabetes association locus among non-HLA genes in Chinese.

• Sun C1, Wei H2, Chen X3, Zhao Z1, Du H4, Song W5, Yang Y6, Zhang M1, Lu W1, Pei Z1, Xi L1, Yan J7, Zhi D1, Cheng R1, Luo F1.
• Meta gene.Meta Gene.2016 May 12;9:120-3. doi: 10.1016/j.mgene.2016.05.003. eCollection 2016.
• Type 1 diabetes (T1D) is an autoimmune disease that has strong contribution of genetic factors to its etiology. We aimed to assess the genetic association between non-HLA genes and T1D in a Chinese case-control cohort recruited from multiple centers consisting of 364 patients with T1D and 719 unrela
• PMID 27331016

Japanese Journal

• 臨床 先天性第XI因子欠乏症患者に発症した舌癌の治療経験

• 耳鼻咽喉科臨床 109(10), 729-734, 2016-10
• NAID 40020970732

• The plasma levels of brain-derived neurotrophic factor are positively associated with emergence agitation in the elderly after gastrointestinal surgery

• Journal of anesthesia 30(5), 811-816, 2016
• NAID 40020963512

• Novel ultra-wideband (UWB) bandpass filter using multiple-mode resonator

• IEICE Electronics Express 13(11), 20160425-20160425, 2016
• NAID 130005156749

Related Links

• factor XI - definition of factor XI by Medical dictionary

factor XI n. A factor in the clotting of blood, a deficiency of which results in a hemorrhagic tendency. Also called plasma thromboplastin antecedent. factor XI, a coagulation factor present in normal plasma. Deficiency results in ...

• Factor XI Deficiency Mutation Database

Factor XI Deficiency mutation database ... Type I(CRM-) is characterized by both low FXI coagulant activity (FXI:C) and low antigen (FXI:Ag). This indicates that the mutant protein is either present in lower amounts or absent in plasma ...

Related Pictures

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★リンクテーブル★

リンク元	「第XI因子」「プラスマトロンボプラスチン前駆物質」「coagulation factor XI」「因子XI」「血漿トロンボプラスチン前駆物質」
拡張検索	「congenital factor XI deficiency」「blood coagulation factor XIII」「blood coagulation factor XI」「factor XII fragment」
関連記事	「fact」「factor」「X」

「第XI因子」

　　[★]

英

factor XI

関

血漿トロンボプラスチン前駆物質、血液凝固第XI因子、凝固第XI因子

「プラスマトロンボプラスチン前駆物質」

　　[★]

英

plasma thromboplastin antecedent, PTA

関

coagulation factors, 第XI因子 factor XI

「coagulation factor XI」

　　[★]

• 凝固第XI因子

関

blood coagulation factor XI、factor XI

「因子XI」

　　[★]

英

[[]]

同

factor XI

関

[[]]

同

factor XI

「血漿トロンボプラスチン前駆物質」

　　[★]

英

factor XI

関

第XI因子

「congenital factor XI deficiency」

　　[★]

先天性第XI因子欠乏症、先天性第XI因子欠損症

関

hemophilia C

「blood coagulation factor XIII」

　　[★]

• 血液凝固第XIII因子

関

coagulation factor XIII、factor XIII

「blood coagulation factor XI」

　　[★]

• 血液凝固第XI因子

関

coagulation factor XI、factor XI

「factor XII fragment」

　　[★] 第XII因子フラグメント

「fact」

　　[★]

• n.

• 事実、現状、実際、実態

関

actual、actually、in fact、in practice、indeed、practically

　 　 　 　 　

「factor」

　　[★]

• n.

• 因子、要因、要素、ファクター

関

element、elementary、factorial、parameter

　 　 　 　 　 　 　

「X」

　　[★] キサントシン, xanthosine