coagulation factor XI

• 凝固第XI因子

関

blood coagulation factor XI、factor XI

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
5. 凝固,凝結

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/08/14 21:40:26」(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
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 • serine-type aminopeptidase activity Cellular component • extracellular region • extracellular space • plasma membrane • membrane Biological process • proteolysis • 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: 187.19 – 187.21 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.

Physiology

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, 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).

Protein structure and molecular biology

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, of both sexes. 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

• Intrinsic pathway
• Extrinsic pathway

References

Further reading

• Gailani D, Zivelin A, Sinha D, Walsh PN (2005). "Do platelets synthesize factor XI?". J. Thromb. Haemost. 2 (10): 1709–12. doi:10.1111/j.1538-7836.2004.00935.x. PMID 15456479. 
• Dossenbach-Glaninger A, Hopmeier P (2005). "Coagulation factor XI: a database of mutations and polymorphisms associated with factor XI deficiency". Blood Coagul. Fibrinolysis 16 (4): 231–8. doi:10.1097/01.mbc.0000169214.62560.a5. PMID 15870541. 
• Seligsohn U (2007). "Factor XI in haemostasis and thrombosis: past, present and future". Thromb. Haemost. 98 (1): 84–9. PMID 17597996. 
• Meijers JC, Davie EW, Chung DW (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 (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 et al. (1991). "A detailed multipoint map of human chromosome 4 provides evidence for linkage heterogeneity and position-specific recombination rates". Am. J. Hum. Genet. 48 (5): 911–25. PMC 1683054. PMID 1673289. 
• Bodfish P, Warne D, Watkins C et al. (1992). "Dinucleotide repeat polymorphism in the human coagulation factor XI gene, intron B (F11), detected using the polymerase chain reaction". Nucleic Acids Res. 19 (24): 6979. doi:10.1093/nar/19.24.6979-a. PMC 329377. PMID 1762944. 
• Clarkson K, Rosenfeld B, Fair J et al. (1991). "Factor XI deficiency acquired by liver transplantation". Ann. Intern. Med. 115 (11): 877–9. PMID 1952475. 
• McMullen BA, Fujikawa K, Davie EW (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 (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". J. Biol. Chem. 266 (12): 7353–8. PMID 2019570. 
• Asakai R, Chung DW, Davie EW, Seligsohn U (1991). "Factor XI deficiency in Ashkenazi Jews in Israel". N. Engl. J. Med. 325 (3): 153–8. doi:10.1056/NEJM199107183250303. PMID 2052060. 
• España F, Berrettini M, Griffin JH (1989). "Purification and characterization of plasma protein C inhibitor". Thromb. Res. 55 (3): 369–84. doi:10.1016/0049-3848(89)90069-8. PMID 2551064. 
• Asakai R, Chung DW, Ratnoff OD, Davie EW (1989). "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 (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 (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". Biochem. Biophys. Res. Commun. 133 (2): 417–22. doi:10.1016/0006-291X(85)90922-2. PMID 3936495. 
• Bouma BN, Vlooswijk RA, Griffin JH (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 et al. (1982). "Factor XI antigen and activity in human platelets". Blood 59 (6): 1148–56. PMID 7044446. 
• Imanaka Y, Lal K, Nishimura T et al. (1995). "Identification of two novel mutations in non-Jewish factor XI deficiency". Br. J. Haematol. 90 (4): 916–20. doi:10.1111/j.1365-2141.1995.tb05215.x. PMID 7669672. 
• Pugh RE, McVey JH, Tuddenham EG, Hancock JF (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 (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 (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 USA 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. 後天性の凝固因子阻害物質 acquired inhibitors of coagulation
• 2. 第ⅩＩ因子欠乏症 factor xi deficiency
• 3. 静脈血栓症の原因の概要 overview of the causes of venous thrombosis
• 4. 止血の概要 overview of hemostasis
• 5. 稀な遺伝性凝固異常 rare inherited coagulation disorders

English Journal

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• Gene expression changes in human mesenchymal stem cells from patients with osteoporosis.

• Liu L1, Zhu Q2, Wang J1, Xi Q1, Zhu H1, Gu M1.
• Molecular medicine reports.Mol Med Rep.2015 Jul;12(1):981-7. doi: 10.3892/mmr.2015.3514. Epub 2015 Mar 19.
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• Zhao YF1, Zhang Q2, Xi JY3, Li YH4, Ma CG5, Xiao BG6.
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• PMID 25908255

Japanese Journal

• Activity of selected coagulation factors in overt and subclinical hypercortisolism

• Endocrine Journal 62(8), 687-694, 2015
• NAID 130005094621

• 第IX・XI・XII因子低下を伴う高齢者後天性血友病A

• 日本老年医学会雑誌 52(3), 285-290, 2015
• NAID 130005093069

• 凍結融解を繰り返した400ml全血採血由来新鮮凍結血漿の品質について

• 日本輸血細胞治療学会誌 61(3), 403-408, 2015
• NAID 130005088381

Related Links

• OMIM Entry - * 264900 - COAGULATION FACTOR XI; F11

TEXT Description Factor XI is a glycoprotein that circulates in plasma as a noncovalent complex with high molecular weight kininogen (HMWK; see 612358). It ...

• F11 - Coagulation factor XI precursor - Homo sapiens (Human ...

Factor XI triggers the middle phase of the intrinsic pathway of blood coagulation by activating factor IX. ... Disease description A hemorrhagic disease characterized by reduced levels and activity of factor XI resulting in moderate ...

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★リンクテーブル★

リンク元	「凝固第XI因子」
拡張検索	「blood coagulation factor XIII」「coagulation factor XIII」「blood coagulation factor XI」「coagulation factor XII」
関連記事	「fact」「factor」「coagulation factor」「X」「factor XI」

「凝固第XI因子」

　　[★]

英

coagulation factor XI

関

第XI因子、血液凝固第XI因子

「blood coagulation factor XIII」

　　[★]

• 血液凝固第XIII因子

関

coagulation factor XIII、factor XIII

「coagulation factor XIII」

　　[★]

• 凝固第XIII因子

関

blood coagulation factor XIII、factor XIII

「blood coagulation factor XI」

　　[★]

• 血液凝固第XI因子

関

coagulation factor XI、factor XI

「coagulation factor XII」

　　[★]

• 凝固第XII因子

関

factor XII、Hageman factor

「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

　 　 　 　 　 　 　

「coagulation factor」

　　[★] 血液凝固因子

同

coagulation factors

「X」

　　[★] キサントシン, xanthosine 　 　

「factor XI」

　　[★]

同

plasma thromboplastin antecedent