関: coagulation factors, factor XII

同: factor XII

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be a contributing factor; "make things factor into a companys profitability"
any of the numbers (or symbols) that form a product when multiplied together
an independent variable in statistics
anything that contributes causally to a result; "a number of factors determined the outcome"
consider as relevant when making a decision; "You must factor in the recent developments" (同)factor in, factor out
resolve into factors; "a quantum computer can factor the number 15" (同)factor in, factor out
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"
a concept whose truth can be proved; "scientific hypotheses are not facts"
a piece of information about circumstances that exist or events that have occurred; "first you must collect all the facts of the case"
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"

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〈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

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/11/21 00:48:41」(JST)

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Coagulation factor XII, also known as Hageman factor, is a plasma protein. It is the zymogen form of factor XIIa, an enzyme (EC 3.4.21.38) of the serine protease (or serine endopeptidase) class. In humans, factor XII is encoded by the F12 gene.^[1]

Structure

Human Factor XII is 596 amino acids long and consists of two domains, the heavy chain (353 residues) and light chain (243 residues) held together by a disulfide bond. It is 80,000 daltons. Its heavy chain contains two fibronectin-type domains (type I and II), two epidermal growth factor-like domains, a kringle domain, and a proline-rich region, and its light chain contains the protease domain. Recently, the structure of the FnI-EGF-like tandem domain of coagulation factor XII was solved by x-ray crystallography.^[2]^[3]

Function

Factor XII is part of the coagulation cascade and activates factor XI and prekallikrein in vitro. Factor XII itself is activated to factor XIIa by negatively charged surfaces, such as glass. This is the starting point of the intrinsic pathway, which is now regarded as an obsolete term, because of the fact that there is no intrinsic pathway in vivo. Factor XII is only used to start coagulation cascades in laboratory studies and is considered to have little to no effect on coagulation in vivo.^[4]

The coagulation cascade.

In vivo, factor XII is activated by contact to polyanions. Activated platelets secrete inorganic polymers, polyphosphates. Contact to polyphosphates activates factor XII and initiates fibrin formation by the intrinsic pathway of coagulation with critical importance for thrombus formation. Targeting polyphosphates with phosphatases interfered with procoagulant activity of activated platelets and blocked platelet-induced thrombosis in mice. Addition of polyphosphates restored defective plasma clotting of Hermansky-Pudlak syndrome patients, indicating that the inorganic polymer is the endogenous factor XII activator in vivo. Platelet polyphosphate-driven factor XII activation provides the link from primary hemostasis (formation of a platelet plug) to secondary hemostasis (fibrin meshwork formation).^[5]

Genetics

The gene for factor XII is located on the tip of the long arm of the fifth chromosome (5q33-qter).^[1]

Role in disease

Factor XII deficiency is a rare disorder that is inherited in an autosomal recessive manner.^[6] Unlike other clotting factor deficiencies, factor XII deficiency is totally asymptomatic and does not cause excess bleeding.^[6] Mice lacking the gene for factor XII, however, are less susceptible to thrombosis. The protein seems to be involved in the later stages of clot formation rather than the first occlusion of damages in the blood vessel wall.^[7]

Factor XII does play an important role in clot formation during in vitro measurements of the partial thromboplastin time, which causes these measurements to be markedly prolonged in patients with factor XII deficiency, usually well beyond even what is seen in hemophilia A, hemophilia B, or factor XI deficiency.^[6] As a result, the main concern related to factor XII deficiency is the unnecessary testing, delay in care, worry, etc. that may be prompted by the abnormal lab result.^[6] All of this, including the mechanism of inheritance, also holds true for the other contact factors, prekallikrein (Fletcher factor) and high molecular weight kininogen.^[6]

Excess levels of factor XII can predispose individuals towards greater risk of venous thrombosis due to factor XII's role as one of the catalysts for conversion of plasminogen to its active fibrinolytic form of plasmin.^[8]

Factor XII is also activated by endotoxins, especially lipid A.

History

Hageman factor was first discovered in 1955 when a routine preoperative blood sample of the 37-year-old railroad brakeman John Hageman (1918) was found to have prolonged clotting time in test tubes, even though he had no hemorrhagic symptoms. Hageman was then examined by Dr. Oscar Ratnoff, who found that Mr. Hageman lacked a previously unidentified clotting factor.^[9] Dr. Ratnoff later found that the Hageman factor deficiency is an autosomal recessive disorder, after examining several related people who had the deficiency. Paradoxically, pulmonary embolism contributed to Hageman's death after an occupational accident. Since then, case studies and clinical studies identified an association between thrombosis and Factor XII deficiency. Hepatocytes express blood coagulation factor XII.^[10]

References

^ ^a ^b Cool DE, MacGillivray RT (October 1987). "Characterization of the human blood coagulation factor XII gene. Intron/exon gene organization and analysis of the 5'-flanking region". J. Biol. Chem. 262 (28): 13662–73. PMID 2888762.
^ Stavrou E, Schmaier AH (March 2010). "Factor XII: what does it contribute to our understanding of the physiology and pathophysiology of hemostasis & thrombosis". Thromb. Res. 125 (3): 210–5. doi:10.1016/j.thromres.2009.11.028. PMC 2851158. PMID 20022081.
^ Beringer DX, Kroon-Batenburg LM (February 2013). "The structure of the FnI-EGF-like tandem domain of coagulation factor XII solved using SIRAS". Acta Crystallogr. Sect. F Struct. Biol. Cryst. Commun. 69 (Pt 2): 94–102. doi:10.1107/S1744309113000286. PMID 23385745.
^ Renné T, Schmaler AH, Nickel KF, Blombäck M, Maas C (2012). "In vivo roles of factor XII.". Blood 120 (22): 4296–303. doi:10.1182/blood-2012-07-292094. PMID 22993391.
^ Müller F, Mutch, NJ, Schenk WA, Smith SA, Esterl L, Spronk HM, Schmidbauer S, Gahl WA, Morrissey JH, Renné T (Dec 2009). "Platelet polyphosphates are proinflammatory and procoagulant mediators in vivo.". CELL 139 (6): 1143–56. doi:10.1016/j.cell.2009.11.001. PMC 2796262. PMID 20005807.
^ ^a ^b ^c ^d ^e Wagenman, BL; Townsend, KT, Mathew, P, Crookston, KP (June 2009). "The laboratory approach to inherited and acquired coagulation factor deficiencies.". Clinics in laboratory medicine 29 (2): 229–52. doi:10.1016/j.cll.2009.04.002. PMID 19665676. Cite uses deprecated parameters (help); |accessdate= requires |url= (help)
^ Renné T, Pozgajová M, Grüner S, Schuh K, Pauer H-U, Burfeind P, Gailani D, Nieswandt B (Jul 2005). "Defective thrombus formation in mice lacking coagulation factor XII.". The Journal of Experimental Medicine 202 (2): 271–81. doi:10.1084/jem.20050664. PMC 2213000. PMID 16009717.
^ Kroll, Michael H. (2001). Manual of Coagulation Disorders. Blackwell Science. pp. 3–4, 206–207. ISBN 0-86542-446-2.
^ Ratnoff OD, Margolius A (1955). "Hageman trait: an asymptomatic disorder of blood coagulation". Trans. Assoc. Am. Physicians 68: 149–54. PMID 13299324.
^ Gordon EM, Gallagher CA, Johnson TR, Blossey BK, Ilan J (April 1990). "Hepatocytes express blood coagulation factor XII (Hageman factor)". J. Lab. Clin. Med. 115 (4): 463–9. PMID 2324612.

External links

The MEROPS online database for peptidases and their inhibitors: S01.211
Factor XII at the US National Library of Medicine Medical Subject Headings (MeSH)

UpToDate Contents

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1. 静脈血栓症の原因の概要 overview of the causes of venous thrombosis
2. 止血の概要 overview of hemostasis
3. 第ⅩＩ因子欠乏症 factor xi deficiency
4. 凝固検査の臨床使用 clinical use of coagulation tests
5. C1インヒビター正常を示す遺伝性血管性浮腫（III型HAE） hereditary angioedema with normal c1 inhibitor type iii hae

English Journal

[Acute ischemic stroke : New approaches to antithrombotic treatment.]

Kraft P, Nieswandt B, Stoll G, Kleinschnitz C.SourceNeurologische Klinik und Poliklinik, Universitatsklinik Wurzburg, Josef-Schneider Str. 11, 97080, Wurzburg, Deutschland.
Der Nervenarzt.Nervenarzt.2011 Sep 8. [Epub ahead of print]
The only recommended therapy in the acute phase of ischemic stroke is thrombolysis within 4.5-(6) h after symptom onset. For secondary stroke prevention platelet inhibitors or, in cases of cardiac embolism, anticoagulants are used. However, these substances bear significant limitations: either they
PMID 21901539

Basement membrane changes in capillaries of the ageing human retina.

Powner MB, Scott A, Zhu M, Munro PM, Foss AJ, Hageman GS, Gillies MC, Fruttiger M.SourceDepartment of Cell Biology, UCL Institute of Ophthalmology, 11-43 Bath Street, London EC1V 9EL, UK; m.fruttiger@ucl.ac.uk.
The British journal of ophthalmology.Br J Ophthalmol.2011 Sep;95(9):1316-22. Epub 2011 May 23.
Objectives The ultrastructural appearance of retinal capillaries can yield important information about disease mechanisms, but is not well characterised in human post mortem samples. We therefore aimed to create a baseline for the appearance of capillaries and establish how this is influenced by pos
PMID 21606466

Japanese Journal

第12因子(ハーゲマン因子) (広範囲血液・尿化学検査免疫学的検査(第7版・2)その数値をどう読むか) -- (血液凝固・線溶系検査)

齋藤英彦
日本臨床 68(-) (970), 722-724, 2010-01
NAID 40016932940

飼育下マイルカ科鯨類4種及びキタオットセイの血液凝固に関する測定値

香山薫,米崎史郎
哺乳類科学 = Mammalian Science 47(2), 221-225, 2007-12-30
… 測定を実施した鯨目全種において，血液凝固に関するスクリーニングテストで，ヒトと比較して活性化部分トロンボプラスチン時間（APTT）の顕著な延長が認められ，血液凝固因子活性の測定では第XII因子（Hageman因子）活性の顕著な低値が確認された．これらが，鯨類の血液凝固時間の延長要因に関係すると考えられる．一方，血液凝固時間の延長のない食肉類のキタオットセイでは，鯨類やヒトと異なる結果が得ら …
NAID 10020029180

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★リンクテーブル★

リンク元	「血液凝固因子」「第XII因子」「coagulation factor XII」
拡張検索	「Hageman factor deficiency」「activated Hageman factor」
関連記事	「fact」「factor」

「血液凝固因子」

Coagulation factor XII (Hageman factor)
Available structures
PDB	Ortholog search: PDBe, RCSB
List of PDB id codes
	4BDW, 4BDX
Identifiers
Symbols	F12 ; HAE3; HAEX; HAF
External IDs	OMIM: 610619 MGI: 1891012 HomoloGene: 425 ChEMBL: 2821 GeneCards: F12 Gene
EC number	3.4.21.38
Gene ontology
Molecular function	• serine-type endopeptidase activity; • protein binding; • misfolded protein binding; • serine-type aminopeptidase activity;
Cellular component	• extracellular region; • extracellular space; • plasma membrane; • extracellular vesicular exosome;
Biological process	• plasma kallikrein-kinin cascade; • Factor XII activation; • proteolysis; • blood coagulation; • blood coagulation, intrinsic pathway; • positive regulation of plasminogen activation; • protein processing; • protein autoprocessing; • positive regulation of blood coagulation; • zymogen activation; • fibrinolysis; • innate immune response; • response to misfolded protein; • positive regulation of fibrinolysis;
	Sources: Amigo / QuickGO
RNA expression pattern
	More reference expression data
Orthologs
	This box: view; talk; edit;

　　[★]

英: coagulation factor (Z), clotting factor, blood coagulation factor, blood coagulation factors
関: ビタミンK

血液凝固因子

表

PT.261

型	名称	同義語	分子量 (kDa)	血漿中濃度 (μg/ml)	半減期 (day)
内因系のプロ酵素	XII	Hageman因子	80	29	2
	プレカリクレイン	Fletcher因子	88	45
	XI	PTA	160	4	2.5
ビタミンK依存	VII	proconvertin	50	0.5	0.2
凝固プロ酵素	IX	Christmas因子	57	4	1
	X	Stuart因子	57	8	1.5
	II	プロトロンビン	70	150	3
補助因子	III	組織因子		0
	血小板ホスホリピド
	高分子量キニノゲン		120	70
	V	proaccelerin	330	7	1.5
	VIII	抗血友病因子			0.5
フィブリン堆積因子	I	フィブリノゲン	340	2500	4.5
	XIII	フィブリン安定因子	320	8	7
抑制物質	アンチトロンビンIII		58	150	2.5

標準血液病学 p.217

15種類ある

凝固因子	慣用語・同義語	ビタミンK依存	血液中半減期(hr)	産生器官	染色体座乗部位
I	fibrinogen		100-150	肝臓	4q26
II	prothronbin	○	50-80	肝臓	11p141-q12
III	tissue thromboplastin			体の各組織	1pter-p21
IV	calcium ion			肝臓
V	labile factor/proaccelerin		24	肝臓	1q21-q25
VII	stable factor/proconvertin	○	6	肝臓	13q34
VIII	antihemophilic factor		12	細網系?	xq28
IX	Christmas factor	○	24	肝臓	xq26.3-q27.2
X	Stuart factor	○	25-60	肝臓	13q34
XI	plasma thromboplastin antecedent		40-80	肝臓	4q35
XII	Hageman factor		50-70	肝臓	5q33-qter
XIII	fibrin stabilizing factor		150	肝臓	6p24-p21.3
prekallikrein	Fletcher factor		35	肝臓	4q35
hight molecular weight kininogen	Fitzgerold factor		150	肝臓	3q26-qter
von Willebrand factor			24	血管内皮血小板糸球体	12pter-p12