同: proaccelerin／labile factor／accelerator globulin

WordNet

a coagulation factor (同)prothrombin accelerator, accelerator factor, factor_V
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"
the 22nd letter of the Roman alphabet (同)v

PrepTutorEJDIC

(…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
〈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
vanadium の化学記号
verb / verso / 《話》very / velocity / volt[s] / volume / vide

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/19 21:17:17」(JST)

wiki en

Factor V (pronounced factor five) is a protein of the coagulation system, rarely referred to as proaccelerin or labile factor. In contrast to most other coagulation factors, it is not enzymatically active but functions as a cofactor. Deficiency leads to predisposition for hemorrhage, while some mutations (most notably factor V Leiden) predispose for thrombosis.

Genetics

The gene for factor V is located on the first chromosome (1q23). It is genomically related to the family of multicopper oxidases, and is homologous to coagulation factor VIII. The gene spans 70 kb, consists of 25 exons, and the resulting protein has a relative molecular mass of approximately 330kDa.

Physiology

Factor V synthesis occurs in the liver, principally. The molecule circulates in plasma as a single-chain molecule with a plasma half-life of 12–36 hours.^[1]

Factor V is able to bind to activated platelets and is activated by thrombin. On activation, factor V is spliced in two chains (heavy and light chain with molecular masses of 110000 and 73000, respectively) which are noncovalently bound to each other by calcium. The thereby activated factor V (now called FVa) is a cofactor of the prothrombinase complex: The activated factor X (FXa) enzyme requires calcium and activated factor V to convert prothrombin to thrombin on the cell surface membrane.

Factor Va is degraded by activated protein C, one of the principal physiological inhibitors of coagulation. In the presence of thrombomodulin, thrombin acts to decrease clotting by activating Protein C; therefore, the concentration and action of protein C are important determinants in the negative feedback loop through which thrombin limits its own activation.

Role in disease

Various hereditary disorders of factor V are known. Deficiency is associated with a rare mild form of hemophilia (termed parahemophilia or Owren parahemophilia), the incidence of which is about 1:1,000,000. It inherits in an autosomal recessive fashion.

Other mutations of factor V are associated with venous thrombosis. They are the most common hereditary causes for thrombophilia (a tendency to form blood clots). The most common one of these, factor V Leiden, is due to the replacement of an arginine residue with glutamine at amino acid position 506 (R506Q). All prothrombotic factor V mutations (factor V Leiden, factor V Cambridge, factor V Hong Kong) make it resistant to cleavage by activated protein C ("APC resistance"). It therefore remains active and increases the rate of thrombin generation.

Laboratory findings in various platelet and coagulation disorders (V - T)
Condition	Prothrombin time	Partial thromboplastin time	Bleeding time	Platelet count
Vitamin K deficiency or warfarin	Prolonged	Normal or mildly prolonged	Unaffected	Unaffected
Disseminated intravascular coagulation	Prolonged	Prolonged	Prolonged	Decreased
Von Willebrand disease	Unaffected	Prolonged or unaffected	Prolonged	Unaffected
Hemophilia	Unaffected	Prolonged	Unaffected	Unaffected
Aspirin	Unaffected	Unaffected	Prolonged	Unaffected
Thrombocytopenia	Unaffected	Unaffected	Prolonged	Decreased
Liver failure, early	Prolonged	Unaffected	Unaffected	Unaffected
Liver failure, end-stage	Prolonged	Prolonged	Prolonged	Decreased
Uremia	Unaffected	Unaffected	Prolonged	Unaffected
Congenital afibrinogenemia	Prolonged	Prolonged	Prolonged	Unaffected
Factor V deficiency	Prolonged	Prolonged	Unaffected	Unaffected
Factor X deficiency as seen in amyloid purpura	Prolonged	Prolonged	Unaffected	Unaffected
Glanzmann's thrombasthenia	Unaffected	Unaffected	Prolonged	Unaffected
Bernard-Soulier syndrome	Unaffected	Unaffected	Prolonged	Decreased or unaffected
Factor XII deficiency	Unaffected	Prolonged	Unaffected	Unaffected
C1INH deficiency	Unaffected	Shortened	Unaffected	Unaffected

History

Until the discovery of factor V, coagulation was regarded as a product of four factors: calcium (IV) and thrombokinase (III) together acting on prothrombin (II) to produce fibrinogen (I); this model had been outlined by Paul Morawitz in 1905.^[2]

The suggestion that an additional factor might exist was made by Dr Paul Owren (1905–1990), a Norwegian physician, during his investigations into the bleeding tendency of a lady called Mary (1914–2002). She had suffered from nosebleeds and menorrhagia (excessive menstrual blood loss) for most her life, and was found to have a prolonged prothrombin time, suggesting either vitamin K deficiency or chronic liver disease leading to prothrombin deficiency. However, neither were the case, and Owren demonstrated this by correcting the abnormality with plasma from which prothrombin had been removed. Using Mary's serum as index, he found that the "missing" factor, which he labeled V (I-IV having been used in Morawitz' model), had particular characteristics. Most investigations were performed during the Second World War, and while Owren published his results in Norway in 1944, he could not publish them internationally until the war was over. They appeared finally in The Lancet in 1947.^[2]^[3]

The possibility of an extra coagulation factor was initially resisted on methodological grounds by Drs Armand Quick and Walter Seegers, both world authorities in coagulation. Confirmatory studies from other groups led to their final approval several years later.^[2]

Owren initially felt that factor V (labile factor or proaccelerin) activated another factor, which he named VI. VI was the factor that accelerated the conversion from prothrombin to thrombin. It was later discovered that factor V was "converted" (activated) by thrombin itself, and later still that factor VI was simply the activated form of factor V.^[2]

The complete amino acid sequence of the protein was published in 1987.^[4] In 1994 factor V Leiden, resistant to inactivation by protein C, was described; this abnormality is the most common genetic cause for thrombosis.^[5]

Interactions

Factor V has been shown to interact with Protein S.^[6]^[7]

References

^ Huang JN, Koerper MA (Nov 2008). "Factor V deficiency: a concise review". Haemophilia 14 (6): 1164–1169. doi:10.1111/j.1365-2516.2008.01785.x. PMID 19141156.
^ ^a ^b ^c ^d Stormorken H (Feb 2003). "The discovery of factor V: a tricky clotting factor". Journal of Thrombosis and Haemostasis 1 (2): 206–13. doi:10.1046/j.1538-7836.2003.00043.x. PMID 12871488.
^ Owren PA (Apr 1947). "Parahaemophilia; haemorrhagic diathesis due to absence of a previously unknown clotting factor". Lancet 1 (6449): 446–51. doi:10.1016/S0140-6736(47)91941-7. PMID 20293060.
^ Jenny RJ, Pittman DD, Toole JJ, Kriz RW, Aldape RA, Hewick RM et al. (Jul 1987). "Complete cDNA and derived amino acid sequence of human factor V". Proceedings of the National Academy of Sciences of the United States of America 84 (14): 4846–50. doi:10.1073/pnas.84.14.4846. PMC 305202. PMID 3110773.
^ Bertina RM, Koeleman BP, Koster T, Rosendaal FR, Dirven RJ, de Ronde H et al. (May 1994). "Mutation in blood coagulation factor V associated with resistance to activated protein C". Nature 369 (6475): 64–7. doi:10.1038/369064a0. PMID 8164741.
^ Heeb MJ, Kojima Y, Rosing J, Tans G, Griffin JH (Dec 1999). "C-terminal residues 621-635 of protein S are essential for binding to factor Va". The Journal of Biological Chemistry 274 (51): 36187–92. doi:10.1074/jbc.274.51.36187. PMID 10593904.
^ Heeb MJ, Mesters RM, Tans G, Rosing J, Griffin JH (Feb 1993). "Binding of protein S to factor Va associated with inhibition of prothrombinase that is independent of activated protein C". The Journal of Biological Chemistry 268 (4): 2872–7. PMID 8428962.

External links

The Coagulation Factor V Protein

UpToDate Contents

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1. 第V因子ライデンおよび活性化プロテインC抵抗性：臨床症状および診断 factor v leiden and activated protein c resistance clinical manifestations and diagnosis
2. 先天性血栓性素因のマネージメント management of inherited thrombophilia
3. 稀な（劣性遺伝性）凝固異常 rare recessively inherited coagulation disorders
4. 後天性の凝固因子阻害物質 acquired inhibitors of coagulation
5. 無症候性患者における先天性血栓性素因のスクリーニング screening for inherited thrombophilia in asymptomatic individuals

English Journal

Thrombophilia testing in children: A 7 year experience.

Mahajerin A, Obasaju P, Eckert G, Vik TA, Mehta R, Heiny M.Author information Department of Pediatrics, Riley Hospital for Children at Indiana University Health, Indiana University School of Medicine, Indianapolis, Indiana.AbstractBACKGROUND: Incidence of venous thromboembolism (VTE) in children is reported to be increasing. We examined thrombophilia testing results in children with VTE that presented in inpatient and outpatient settings to explore patterns of thrombophilia testing.
Pediatric blood & cancer.Pediatr Blood Cancer.2014 Mar;61(3):523-7. doi: 10.1002/pbc.24846. Epub 2013 Nov 19.
BACKGROUND: Incidence of venous thromboembolism (VTE) in children is reported to be increasing. We examined thrombophilia testing results in children with VTE that presented in inpatient and outpatient settings to explore patterns of thrombophilia testing.PROCEDURE: Patients/Methods: Children, ages
PMID 24249220

Combined genetic mutations have remarkable effect on deep venous thrombosis and/or pulmonary embolism occurence.

Simsek E1, Yesilyurt A2, Pinarli F3, Eyerci N4, Ulus AT5.Author information 1Turkiye Yuksek Ihtisas Training and Research Hospital, Department of Cardiovascular Surgery, Ankara, Turkey. Electronic address: erdaldr@yahoo.com.2Diskapi Yildirim Beyazit Training and Research Hospital, Department of Stem Cell and Genetic Diagnostic Center, Diskapi, Ankara, Turkey. Electronic address: dryesilyurt@hotmail.com.3Diskapi Yildirim Beyazit Training and Research Hospital, Department of Stem Cell and Genetic Diagnostic Center, Diskapi, Ankara, Turkey. Electronic address: ferdapinarli@yahoo.com.4Diskapi Yildirim Beyazit Training and Research Hospital, Department of Stem Cell and Genetic Diagnostic Center, Diskapi, Ankara, Turkey. Electronic address: helezon2003@gmail.com.5Turkiye Yuksek Ihtisas Training and Research Hospital, Department of Cardiovascular Surgery, Ankara, Turkey. Electronic address: uluss@yahoo.com.AbstractPURPOSE: Although deep vein thrombosis and thromboembolic diseases differ among various races, they are still important in our day. The difficulties in treatment and following-up of these diseases are caused by secret genetic mutations rather than predisposing factors.
Gene.Gene.2014 Feb 15;536(1):171-6. doi: 10.1016/j.gene.2013.11.019. Epub 2013 Dec 12.
PURPOSE: Although deep vein thrombosis and thromboembolic diseases differ among various races, they are still important in our day. The difficulties in treatment and following-up of these diseases are caused by secret genetic mutations rather than predisposing factors.METHODS: Between January 2011 a
PMID 24334115

The relationship between factor V Leiden, prothrombin G20210A, and MTHFR mutations and the first major thrombotic episode in polycythemia vera and essential thrombocythemia.

Trifa AP, Cucuianu A, Popp RA, Coadă CA, Costache RM, Militaru MS, Vesa SC, Pop IV.Author information Department of Medical Genetics, "Iuliu Haţieganu" University of Medicine and Pharmacy, 6 Pasteur St, 400349, Cluj-Napoca, Romania, trifa.adrian@umfcluj.ro.AbstractArterial and venous thrombosis are the most frequent complications in patients with polycythemia vera and essential thrombocythemia. We sought to demonstrate a possible contribution of the factor V Leiden, prothrombin G20210A, and methylenetetrahydrofolate reductase (MTHFR) 677 C > T and 1298 A > C mutations to the thrombotic risk in patients with polycythemia vera and essential thrombocythemia along with other biological features of these patients. We included 86 patients with polycythemia vera, of which 34 (39.5 %) had major thrombosis and 95 patients with essential thrombocythemia, of which 22 (23.1 %) had major thrombosis. In the whole cohort of patients, only the factor V Leiden mutation was significantly associated with both arterial and venous thrombosis in univariate and multivariate analysis (odds ratio (OR) = 4.3; 95 % confidence interval (CI) = 1.5-12.5; p = 0.008 and OR = 4.3; 95 % CI = 1.2-15.9; p = 0.02, respectively). Other factors significantly associated with thrombosis in both univariate and multivariate analysis were male sex (OR = 2.8, 95 % CI = 1.4-5.4, p = 0.002 and OR = 3.5, 95 % CI = 1.6-7.6, p = 0.002, respectively) and the JAK2 V617F mutation (OR = 5.5, 95 % CI = 2.1-15, p = 0.0001 and OR = 6.9, 95 % CI = 2.2-21.2, p = 0.001, respectively). In conclusion, among the four mutations analyzed (factor V Leiden, prothrombin G20210A, and MTHFR 677 C > T and 1298 A > C), only factor V Leiden is a major contributor to thrombosis in polycythemia vera and essential thrombocythemia.
Annals of hematology.Ann Hematol.2014 Feb;93(2):203-9. doi: 10.1007/s00277-013-1838-6. Epub 2013 Jul 5.
Arterial and venous thrombosis are the most frequent complications in patients with polycythemia vera and essential thrombocythemia. We sought to demonstrate a possible contribution of the factor V Leiden, prothrombin G20210A, and methylenetetrahydrofolate reductase (MTHFR) 677 C > T and 1298
PMID 23828072

Japanese Journal

Long-term trends of sulfur deposition in East Asia during 1981–2005

Kuribayashi Masatoshi,Ohara Toshimasa,Morino Yu,Uno Itsushi,Kurokawa Jun-ichi,Hara Hiroshi,栗林正俊
Atmospheric environment 59, 461-475, 2012-11
… Sulfur flux from China to Japan increased by a factor of 2.5 at altitudes of 0–3000 m from 1981 to 2005. …
NAID 120004288250

Nerve Growth Factor and the Physiology of Pain: Lessons from Congenital Insensitivity to Pain with Anhidrosis

Indo Yasuhiro,インドウヤスヒロ,犬童康弘
Clinical Genetics 82(4), 341-350, 2012-10
… insensitivity to pain with anhidrosis (CIPA) is an autosomal recessivegenetic disorder characterized by insensitivity to pain, anhidrosis (the inability to sweat)and mental retardation.Nerve growth factor (NGF) is a well-known neurotrophic factor essential for thesurvival and maintenance of NGF-dependent neurons, including primary afferentneurons with thin fibers and sympathetic postganglionic neurons, during development.NGF is also considered to be an inflammatory mediator associated with pain, itch andinflammation in …
NAID 120004724836

Related Pictures

★リンクテーブル★

リンク元	「第V因子」「因子V」「proaccelerin」
拡張検索	「coagulation factor VIIIa」「blood coagulation factor VIII」「factor VIIIa」「activated factor V」
関連記事	「fact」「factor」「Vd」「V」

「第V因子」

Coagulation factor V (proaccelerin, labile factor)
	; PDB rendering based on 1czs.
Available structures
PDB	Ortholog search: PDBe, RCSB
List of PDB id codes
	1CZS, 1CZT, 1CZV, 1FV4, 1Y61, 3P6Z, 3P70, 3S9C
Identifiers
Symbols	F5 ; FVL; PCCF; RPRGL1; THPH2
External IDs	OMIM: 612309 MGI: 88382 HomoloGene: 104 IUPHAR: 2606 ChEMBL: 3618 GeneCards: F5 Gene
Gene ontology
Molecular function	• serine-type endopeptidase activity; • copper ion binding; • protein binding;
Cellular component	• extracellular region; • extracellular space; • plasma membrane; • membrane; • platelet alpha granule lumen;
Biological process	• platelet degranulation; • proteolysis; • cell adhesion; • blood coagulation; • blood circulation; • platelet activation;
	Sources: Amigo / QuickGO
RNA expression pattern
	More reference expression data
Orthologs
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