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(physical chemistry) a fourth state of matter distinct from solid or liquid or gas and present in stars and fusion reactors; a gas becomes a plasma when it is heated until the atoms lose all their electrons, leaving a highly electrified collection of nuclei and free electrons; "particles in space exist in the form of a plasma"
a green slightly translucent variety of chalcedony used as a gemstone
the colorless watery fluid of the blood and lymph that contains no cells, but in which the blood cells (erythrocytes, leukocytes, and thrombocytes) are suspended (同)plasm, blood_plasma
preceding in time or order
anything that precedes something similar in time; "phrenology was an antecedent of modern neuroscience" (同)forerunner
a preceding occurrence or cause or event
the referent of an anaphor; a phrase or clause that is referred to by an anaphoric pronoun
an enzyme liberated from blood platelets that converts prothrombin into thrombin as blood starts to clot (同)thrombokinase, factor_III

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(また『blood plasma』)血漿,リンパ漿 / 原形質
先だつ,先行する / (…に)先だつ人(物,事)《+『to』+『名』》 / (文法で,関係詞の)先行詞 / 《複数形で》先祖(ancestors) / 《複数形で》前歴,経歴,素姓

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/03/30 14:33:08」(JST)

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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.^[1]^[2]^[3]^[4]

Function

Factor XI (FXI) is produced by the liver and circulates as a homo-dimer in its inactive form.^[5] 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).^[6]

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.^[2]^[3]

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.^[7]

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.

References

^ 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.
^ ^a ^b Asakai R, Davie EW, Chung DW (Nov 1987). "Organization of the gene for human factor XI". Biochemistry 26 (23): 7221–8. doi:10.1021/bi00397a004. PMID 2827746.
^ ^a ^b Kato A, Asakai R, Davie EW, Aoki N (1989). "Factor XI gene (F11) is located on the distal end of the long arm of human chromosome 4". Cytogenetics and Cell Genetics 52 (1-2): 77–8. doi:10.1159/000132844. PMID 2612218.
^ 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.
^ Wu W, Sinha D, Shikov S, Yip CK, Walz T, Billings PC, Lear JD, Walsh PN (Jul 2008). "Factor XI homodimer structure is essential for normal proteolytic activation by factor XIIa, thrombin, and factor XIa". The Journal of Biological Chemistry 283 (27): 18655–64. doi:10.1074/jbc.M802275200. PMC 2441546. PMID 18441012.
^ Walsh PN (Jul 2001). "Roles of platelets and factor XI in the initiation of blood coagulation by thrombin". Thrombosis and Haemostasis 86 (1): 75–82. PMID 11487044.
^ Bolton-Maggs PH (Jun 1996). "Factor XI deficiency". Baillière's Clinical Haematology 9 (2): 355–68. doi:10.1016/S0950-3536(96)80068-0. PMID 8800510.

External links

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

Molecular and Cellular Biology portal

UpToDate Contents

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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.
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PMID 27331016

Factor XI as a Therapeutic Target.

Gailani D1, Gruber A2.
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PMID 27174099

Expression of fibroblast growth factor 21 in patients with biliary atresia.

Li D1, Lu T1, Shen C1, Liu Y1, Zhang J1, Shan Y1, Luo Y1, Xi Z1, Qiu B1, Chen Q2, Zhang J1, Xia Q3.
Cytokine.Cytokine.2016 Jul;83:13-8. doi: 10.1016/j.cyto.2016.03.003. Epub 2016 Mar 21.
Fibroblast growth factor 21 is a critical circulating adipokine involving in metabolic disorders and various liver diseases. This study was performed to investigate whether FGF21 is also associated with the pathophysiology of biliary atresia. Serum FGF21 levels were measured in 57 BA patients and 20
PMID 27003131

Japanese Journal

Factor XI (plasma thromboplastin antecedent) deficiency in Ashkenazi Jews is a bleeding disorder that can result from three types of point mutations

ASAKAI R
Proc Natl Acad Sci USA 86, 7667-7671, 1989
NAID 80004829794

A Japanese Family of Hereditary Deficiency of Plasma Thromboplastin Antecedent （Factor ⅩⅠ）

Shinohara kenji,Konishi Yasuko,Azuno Yoichi,Yamada Katsunori,Sasaki Hiroko,Kaneko Toshio,Matsumoto Noboru
The bulletin of the Yamaguchi Medical School 32(1), 53-56, 1985-12
… A Japanese family of hereditary deficiency of plasma thromboplastin antecedent (PTA, factor XI ) was reported. … The levels of plasma factor Xiactivity and anitigen of the homozygotes were less than 1% of normal, and those of heterzygotes were approximately half of normal. …
NAID 120000860089

「血液凝固因子」

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
	v; t; e;

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英: coagulation factor (Z), clotting factor, blood coagulation factor, blood coagulation factors
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