factor VII deficiency

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 22nd letter of the Roman alphabet (同)v

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. 〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥
4. vanadium の化学記号
5. Virgin Islands

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/12/26 03:31:03」(JST)

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Coagulation factor VII (serum prothrombin conversion accelerator)
Anchoring of coagulation factor VIIa (PDB 1dan) to the membrane through its Gla domain
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1BF9, 1CVW, 1DAN, 1DVA, 1F7E, 1F7M, 1FAK, 1FF7, 1FFM, 1J9C, 1JBU, 1KLI, 1KLJ, 1NL8, 1O5D, 1QFK, 1W0Y, 1W2K, 1W7X, 1W8B, 1WQV, 1WSS, 1WTG, 1WUN, 1WV7, 1YGC, 1Z6J, 2A2Q, 2AEI, 2AER, 2B7D, 2B8O, 2BZ6, 2C4F, 2EC9, 2F9B, 2FIR, 2FLB, 2FLR, 2PUQ, 2ZP0, 2ZWL, 2ZZU, 3ELA, 3TH2, 3TH3, 3TH4, 4IBL, 4ISH, 4ISI, 4JYU, 4JYV, 4JZD, 4JZE, 4JZF, 4NA9, 4NG9, 4NGA, 4X8S, 4X8T, 4X8U, 4X8V, 4YT6, 4YT7
Identifiers
Symbols	F7 ; SPCA
External IDs	OMIM: 613878 MGI: 109325 HomoloGene: 7710 ChEMBL: 3991 GeneCards: F7 Gene
EC number	3.4.21.21
Gene ontology Molecular function • glycoprotein binding • serine-type endopeptidase activity • receptor binding • calcium ion binding • protein binding • serine-type peptidase activity Cellular component • extracellular region • extracellular space • endoplasmic reticulum lumen • Golgi lumen • plasma membrane • vesicle Biological process • positive regulation of leukocyte chemotaxis • proteolysis • blood coagulation • blood coagulation, extrinsic pathway • circadian rhythm • positive regulation of platelet-derived growth factor receptor signaling pathway • peptidyl-glutamic acid carboxylation • positive regulation of blood coagulation • positive regulation of cell migration • organ regeneration • response to vitamin K • response to estrogen • post-translational protein modification • cellular protein metabolic process • positive regulation of positive chemotaxis • positive regulation of protein kinase B signaling • response to growth hormone Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	2155	14068
Ensembl	ENSG00000057593	ENSMUSG00000031443
UniProt	P08709	P70375
RefSeq (mRNA)	NM_000131	NM_010172
RefSeq (protein)	NP_000122	NP_034302
Location (UCSC)	Chr 13: 113.11 – 113.12 Mb	Chr 8: 13.03 – 13.04 Mb
PubMed search	[1]	[2]

Factor VII (EC 3.4.21.21, blood-coagulation factor VIIa, activated blood coagulation factor VII, formerly known as proconvertin) is one of the proteins that causes blood to clot in the coagulation cascade. It is an enzyme of the serine protease class. A recombinant form of human factor VIIa (eptacog alfa [activated], NovoSeven) has U.S. Food and Drug Administration approval for uncontrolled bleeding in hemophilia patients. It is sometimes used unlicensed in severe uncontrollable bleeding, although there have been safety concerns. A biosimilar form of recombinant activated factor VII (AryoSeven) is also available, but does not play any considerable role in the market.

Physiology

The main role of factor VII (FVII) is to initiate the process of coagulation in conjunction with tissue factor (TF/factor III). Tissue factor is found on the outside of blood vessels - normally not exposed to the bloodstream. Upon vessel injury, tissue factor is exposed to the blood and circulating factor VII. Once bound to TF, FVII is activated to FVIIa by different proteases, among which are thrombin (factor IIa), factor Xa, IXa, XIIa, and the FVIIa-TF complex itself. The complex of factor VIIa with TF catalyzes the conversion of factor IX and factor X into the active proteases, factor IXa and factor Xa, respectively.

The action of the factor is impeded by tissue factor pathway inhibitor (TFPI), which is released almost immediately after initiation of coagulation. Factor VII is vitamin K dependent; it is produced in the liver. Use of warfarin or similar anticoagulants decreases hepatic synthesis of FVII.

Genetics

The gene for factor VII is located on chromosome 13 (13q34).

Role in disease

Deficiency is rare (congenital proconvertin deficiency) and inherits recessively. Factor VII deficiency presents as a hemophilia-like bleeding disorder. It is treated with recombinant factor VIIa (NovoSeven or AryoSeven).

Medical uses

Recombinant factor VIIa, marketed under the trade names AryoSeven and NovoSeven, is used for people with hemophilia (with Factor VIII or IX deficiency) who have developed inhibitors against replacement coagulation factor.

It has also been used in the setting of uncontrollable hemorrhage, but its role in this setting is controversial with insufficient evidence to support its use outside of clinical trials. The first report of its use in hemorrhage was in an Israeli soldier with uncontrollable bleeding in 1999. Risks of its use include an increase in arterial thrombosis.

Recombinant human factor VII while initially looking promising in intracerebral hemorrhage failed to show benefit following further study and this is no longer recommended.

Interactions

Factor VII has been shown to interact with tissue factor.

References

Further reading

• Broze GJ, Majerus PW (1980). "Purification and properties of human coagulation factor VII". J. Biol. Chem. 255 (4): 1242–7. PMID 7354023. 
• Versteeg HH, Peppelenbosch MP, Spek CA (2002). "The pleiotropic effects of tissue factor: a possible role for factor VIIa-induced intracellular signalling?". Thromb. Haemost. 86 (6): 1353–9. PMID 11776298. 
• Golino P (2003). "The inhibitors of the tissue factor:factor VII pathway". Thromb. Res. 106 (3): V257–65. doi:10.1016/S0049-3848(02)00079-8. PMID 12356487. 

External links

• Official website
• The MEROPS online database for peptidases and their inhibitors: S01.215

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. 稀な遺伝性凝固異常 rare inherited coagulation disorders
• 2. 出血性素因を有する成人患者へのアプローチ approach to the adult patient with a bleeding diathesis
• 3. 非血友病患者における遺伝子組換え第VIIa凝固因子の治療使用 therapeutic uses of recombinant coagulation factor viia in non hemophiliacs
• 4. 出血症状を有する小児に対するアプローチ approach to the child with bleeding symptoms
• 5. 小児における鼻出血の疫学および病因 epidemiology and etiology of epistaxis in children

English Journal

• The carboxyl-terminal region is NOT essential for secreted and functional levels of coagulation factor X.

• Branchini A1,2, Baroni M1,2, Burini F1, Puzzo F1, Nicolosi F1, Mari R3, Gemmati D3, Bernardi F1,2, Pinotti M1,2.
• Journal of thrombosis and haemostasis : JTH.J Thromb Haemost.2015 Jun 17. doi: 10.1111/jth.13034. [Epub ahead of print]
• BACKGROUND: The homologous coagulation factor X (FX), VII (FVII), IX (FIX) and protein C (PC) display striking differences in the carboxyl-terminus, with that of FX being the most extended. This region is essential for FVII, FIX and PC secretion.OBJECTIVES: To provide experimental evidence for the r
• PMID 26083275

• Coagulation abnormalities in 5 cats with naturally occurring cytauxzoonosis.

• Conner BJ1, Hanel RM1, Brooks MB2, Cohn LA3, Birkenheuer AJ1.
• Journal of veterinary emergency and critical care (San Antonio, Tex. : 2001).J Vet Emerg Crit Care (San Antonio).2015 Jun 16. doi: 10.1111/vec.12326. [Epub ahead of print]
• OBJECTIVE: To characterize hemostasis and determine if disseminated intravascular coagulation (DIC) is present in cats with cytauxzoonosis.DESIGN: Cross-sectional study.SETTING: University teaching hospital.ANIMALS: Five client-owned cats with cytologic and PCR-confirmed cytauxzoonosis.INTERVENTIONS
• PMID 26082008

Japanese Journal

• A case of sagittal splitting ramus osteotomy and genioplasty in a patient with congenital factor VII deficiency

• Acta Medica Nagasakiensia 60(1), 21-24, 2015-07
• NAID 110009959636

• 複合型の先天性凝固因子欠乏症患者に発症した結腸憩室出血の1例

• 日本消化器外科学会雑誌 48(1), 60-67, 2015
• NAID 130004908154

• 出血性十二指腸gastrointestinal stromal tumorに対して膵頭十二指腸切除を施行した先天性凝固第VII因子欠乏症の1例

• 日本消化器外科学会雑誌 48(1), 23-30, 2015
• NAID 130004908153

Related Links

• Factor VII deficiency: MedlinePlus Medical Encyclopedia

Factor VII (seven) deficiency is a disorder caused by a lack of a protein called factor VII in the blood. It leads to problems with blood clotting (coagulation). ... When you bleed, a series of reactions take place in the body that helps ...

• Factor VII Deficiency - Diseases & Conditions - Medscape ...

Frequency International Hereditary factor VII deficiency is a rare autosomal recessive bleeding disorder first described by Alexander et al in 1951. [1] Prevalence is estimated to be 1 case per 500,000 persons in the ...

Related Pictures

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★リンクテーブル★

リンク元	「第VII因子欠乏症」「第VII因子欠損症」
関連記事	「deficiency」「fact」「factor」「V」

「第VII因子欠乏症」

　　[★]

英

factor VII deficiency

関

第VII因子欠損症

「第VII因子欠損症」

　　[★]

英

factor VII deficiency

関

第VII因子欠乏症

「deficiency」

　　[★]

• n.

• 不足、欠乏、欠失、欠如、欠損、不十分。栄養不足、栄養素欠乏、欠乏症。(遺伝子)(染色体内の)遺伝子欠失
• 欠けているもの、不足している物。不足分。不完全なもの、欠点のあるもの

関

absence, agenesis, dearth, defect, defective, deficient, deficit, delete, deletion, deletional, depletion, deprivation, deprive, lack, miss, missing, morphological defect, paucity, scarce, scarcity, starve

　

「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

　 　 　 　 　 　 　

「V」

　　[★]

• バリン valine
• ビタミン vitamine
• ventilation