第XIII因子。フィブリン安定化因子
WordNet
- 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"
- a white insoluble fibrous protein formed by the action of thrombin on fibrinogen when blood clots; it forms a network that traps red cells and platelets
- causing to become stable; "the family is one of the great stabilizing elements in society" (同)stabilising
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
- (凝固血液中の)線維素,フィブリン
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/06/05 15:24:05」(JST)
[Wiki en表示]
Factor XIII crosslinks fibrin
coagulation factor XIII,
A1 polypeptide |
Identifiers |
Symbol |
F13A1 |
Alt. symbols |
F13A |
Entrez |
2162 |
HUGO |
3531 |
OMIM |
134570 |
RefSeq |
NM_000129 |
UniProt |
P00488 |
Other data |
Locus |
Chr. 6 p24.2-p23 |
coagulation factor XIII,
B polypeptide |
Identifiers |
Symbol |
F13B |
Entrez |
2165 |
HUGO |
3534 |
OMIM |
134580 |
RefSeq |
NM_001994 |
UniProt |
P05160 |
Other data |
Locus |
Chr. 1 q31-q32.1 |
Factor XIII or fibrin stabilizing factor is an enzyme (EC 2.3.2.13) of the blood coagulation system that crosslinks fibrin.
Contents
- 1 Function
- 2 Discovery
- 3 Genetics
- 4 Physiology
- 5 Diagnostic use
- 6 See also
- 7 References
- 8 External links
Function
Factor XIII is a transglutaminase that circulates in the plasma as a heterotetramer of two catalytic A subunits and two carrier B subunits. When thrombin has converted fibrinogen to fibrin, the latter forms a proteinaceous network in which every E-unit is crosslinked to only one D-unit. Factor XIII is activated by thrombin into factor XIIIa; its activation into Factor XIIIa requires calcium as a cofactor. A cleavage by thrombin between residue Arg37 and Gly38 on the N-terminus of the A subunit, leads to the release of the activation peptide (MW 4000 da). In the presence of calcium the carrier subunits dissociate from the catalytic subunits, leading to a 3D change in conformation of factor XIII and hence the exposure of catalytic cysteine residue. Upon activation by thrombin, factor XIIIa acts on fibrin to form γ-glutamyl-Є-lysyl amide cross links between fibrin molecules to form an insoluble clot.[citation needed]
Discovery
FXIII is known also as Laki-Lorand factor, after the scientists who first proposed its existence in 1948.[1] A 2005 conference recommended standardization of nomenclature.[2]
Genetics
Zymogen factor XIII is a 320kDa glycoprotein tetramer consisting of twice two subunits (2 A and 2 B),[2] the genes for which are on different chromosomes:
- A subunit (6p25-p24). The transglutaminase part; this adds an alkyl group to the nitrogen on a glutamine residue, which binds in turn with a lysine on the other chain. The molecular weight of the A chain is approximately 83kDa.
- B subunit (1q31-q32.1). This has no clear enzymatic activity, and may serve as a carrier for the A subunit. The molecular weight of the B chain is approximately 76.5kDa.
Physiology
Typical concentrations of FXIII in plasma is 10 μg/ml (2A2B heterodimer), while the concentration of free B chain is 22 μg/ml. FXIII has a long half life, ranging from 5–9 days. It is present in plasma, platelets, and monocytes, as well as macrophages and bone marrow precursors of these cell types.[2]
A clot that has not been stabilized by FXIIIa is soluble in 5 mol/L urea, while a stabilized clot is resistant to this phenomenon.[1]
Diagnostic use
Factor XIII levels are not measured routinely, but may be considered in patients with an unexplained bleeding tendency. As the enzyme is quite specific for monocytes and macrophages, determination of the presence of factor XIII may be used to identify and classify malignant diseases involving these cells.[2]
See also
- Factor XIII deficiency
- D-dimer
- Coagulation
References
- ^ a b Laki K, Lóránd L (September 1948). "On the Solubility of Fibrin Clots". Science 108 (2802): 280. doi:10.1126/science.108.2802.280. PMID 17842715.
- ^ a b c d Muszbek L, Ariëns RA, Ichinose A (January 2007). "Factor XIII: recommended terms and abbreviations". J. Thromb. Haemost. 5 (1): 181–3. doi:10.1111/j.1538-7836.2006.02182.x. PMID 16938124.
External links
- Factor XIII deficiency at hemophilia.org
- med/3491 at eMedicine
Proteins: coagulation
|
|
Coagulation factors |
Primary hemostasis |
- vWF
- platelet membrane glycoproteins: Ib (A
- B
- IX)
- IIb/IIIa (IIb
- IIIa)
- VI
|
|
Intrinsic pathway |
- HMWK/Bradykinin
- Prekallikrein/Kallikrein
- XII "Hageman"
- XI
- IX
- VIII
|
|
Extrinsic pathway |
|
|
Common pathway |
- X
- V
- II "(Pro)thrombin"
- I "Fibrin"
- Fibrinogen (FGA, FGG)
- XIII
|
|
|
Coagulation inhibitors |
- Antithrombin (inhibits II, IX, X, XI, XII)
- Protein C (inhibits V, VIII)/Protein S (cofactor for protein C)
- Protein Z (inhibits X)
- ZPI (inhibits X, XI)
- TFPI (inhibits III)
|
|
Thrombolysis/fibrinolysis |
- Plasmin
- tPA/urokinase
- PAI-1/2
- α2-AP
- α2-macroglobulin
- TAFI
|
|
Index of cells from bone marrow
|
|
Description |
- Immune system
- Cells
- Physiology
- coagulation
- proteins
- granule contents
- colony-stimulating
- heme and porphyrin
|
|
Disease |
- Red blood cell
- Monocyte and granulocyte
- Neoplasms and cancer
- Histiocytosis
- Symptoms and signs
- Blood tests
|
|
Treatment |
- Transfusion
- Drugs
- thrombosis
- bleeding
- other
|
|
|
Transferases: acyltransferases (EC 2.3)
|
|
2.3.1: other than amino-acyl groups |
- acetyltransferases: Acetyl-Coenzyme A acetyltransferase
- N-Acetylglutamate synthase
- Choline acetyltransferase
- Dihydrolipoyl transacetylase
- Acetyl-CoA C-acyltransferase
- Beta-galactoside transacetylase
- Chloramphenicol acetyltransferase
- N-acetyltransferase
- Serotonin N-acetyl transferase
- HGSNAT
- ARD1A
- Histone acetyltransferase
- palmitoyltransferases: Carnitine O-palmitoyltransferase
- Serine C-palmitoyltransferase
- other: Acyltransferase like 2
- Aminolevulinic acid synthase
- Beta-ketoacyl-ACP synthase
- Glyceronephosphate O-acyltransferase
- Lecithin—cholesterol acyltransferase
- Glycerol-3-phosphate O-acyltransferase
- 1-acylglycerol-3-phosphate O-acyltransferase
- 2-acylglycerol-3-phosphate O-acyltransferase
- ABHD5
|
|
2.3.2: Aminoacyltransferases |
- Gamma-glutamyl transpeptidase
- Peptidyl transferase
- Transglutaminase
- Tissue transglutaminase
- Keratinocyte transglutaminase
- Factor XIII
|
|
2.3.3: converted into alkyl on transfer |
- Citrate synthase
- ATP citrate lyase
- HMG-CoA synthase
- Malate synthase
|
|
- Biochemistry overview
- Enzymes overview
- By EC number: 1.1
- 2
- 3
- 4
- 5
- 6
- 7
- 8
- 10
- 11
- 13
- 14
- 15-18
- 2.1
- 3.1
- 4.1
- 5.1
- 6.1-3
|
|
|
|
UpToDate Contents
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English Journal
- Thrombin generation and fibrin clot formation under hypothermic conditions: An in vitro evaluation of tissue factor initiated whole blood coagulation.
- Whelihan MF1, Kiankhooy A2, Brummel-Ziedins KE3.Author information 1Departments of Biochemistry and Surgery, College of Medicine, University of Vermont, Burlington, VT. Electronic address: Matthew.whelihan@uvm.edu.2Departments of Biochemistry and Surgery, College of Medicine, University of Vermont, Burlington, VT. Electronic address: Armin.Kiankhooy@vtmednet.org.3Departments of Biochemistry and Surgery, College of Medicine, University of Vermont, Burlington, VT. Electronic address: Kathleen.brummel@uvm.edu.AbstractBACKGROUND: Despite trauma-induced hypothermic coagulopathy being familiar in the clinical setting, empirical experimentation concerning this phenomenon is lacking. In this study, we investigated the effects of hypothermia on thrombin generation, clot formation, and global hemostatic functions in an in vitro environment using a whole blood model and thromboelastography, which can recapitulate hypothermia.
- Journal of critical care.J Crit Care.2014 Feb;29(1):24-30. doi: 10.1016/j.jcrc.2013.10.010. Epub 2013 Oct 29.
- BACKGROUND: Despite trauma-induced hypothermic coagulopathy being familiar in the clinical setting, empirical experimentation concerning this phenomenon is lacking. In this study, we investigated the effects of hypothermia on thrombin generation, clot formation, and global hemostatic functions in an
- PMID 24331944
- Factor XIII Val34Leu polymorphism in patients with Cardiac Syndrome X.
- Güler GB, Batgerel U, Güler E, Karaca O, Geçmen C, Güneş HM, Aguş HZ, Esen AM, Türkmen MM.Author information İstinye State Hospital. gamzebabur@hotmail.com.AbstractAims: To examine the frequency of Factor XIII polymorphism among patients with cardiac syndrome X (CSX). Methods: This study was designed as a cross-sectional and observational study. Forty-eight female patients with CSX and 36 controls matched by age, gender, diabetes, and hypertension were studied. Cardiac syndrome X was defined as typical chest pain during rest or effort, abnormal test result for exercise ECG, and presence of angiographically normal epicardial coronary arteries after ruling out inducible spasm. Factor XIII gene polymorphism was investigated by using CVD Strip Assay (ViennaLab Diagnostic GmbH) commercial kit. Results: The frequency of Factor XIII (Val/Leu + Leu/Leu) mutation was significantly higher in patients with CSX (43%) was significantly higher than than in controls (19%) (p = 0.02). Frequency of the Leu allele was significantly higher in the patient group (23.5% vs. 11.1%, p = 0.04). Factor XIII (Val/Leu + Leu/Leu) mutation (p = 0.01, OR = 3.42; 95% CI [1.22-9.58]) and smoking (p = 0.04, OR = 3.33, 95% CI [1.05, 10.58]) were identified as independent predictors of the disease in multivariate regression analysis.Conclusion: This study indicates that there is an evidence for association between factor XIII Val34Leu polymorphism and CSX.
- Cardiology journal.Cardiol J.2014 Jan 10. doi: 10.5603/CJ.a2013.0046. [Epub ahead of print]
- Aims: To examine the frequency of Factor XIII polymorphism among patients with cardiac syndrome X (CSX). Methods: This study was designed as a cross-sectional and observational study. Forty-eight female patients with CSX and 36 controls matched by age, gender, diabetes, and hypertension were studied
- PMID 23677728
- Interaction of factor XIII subunits.
- Katona E, Pénzes K, Csapó A, Fazakas F, Udvardy ML, Bagoly Z, Orosz ZZ, Muszbek L.Author information Clinical Research Center, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary;AbstractCoagulation factor XIII (FXIII) is a heterotetramer consisting of two catalytic A subunits (FXIII-A2) and two protective/inhibitory B subunits (FXIII-B2). FXIII-B, a mosaic protein consisting of ten sushi domains, significantly prolongs the lifespan of catalytic subunits in the circulation and prevents their slow progressive activation in plasmatic conditions. In this study the biochemistry of the interaction between the two FXIII subunits was investigated. Using surface plasmon resonance technique and an ELISA-type binding assay the equilibrium dissociation constant (Kd) for the interaction was established in the range of 10-10 M. Based on the measured Kd, it was calculated that in plasma approximately 1% of FXIII-A2 should be in free form. This value was confirmed experimentally by measuring FXIII-A2 in plasma samples immuno-depleted of FXIII-A2B2. Free plasma FXIII-A2 became functionally active, when activated by thrombin and Ca2+, it cross-linked fibrin. In cerebrospinal fluid and tears with much lower FXIII subunit concentrations higher than 80% of FXIII-A2 existed in free form. A monoclonal anti-FXIII-B antibody that prevented the interaction between the two subunits reacted with the recombinant combined 1st and 2nd sushi domains of FXIII-B and its epitope was localized to the peptide spanning positions 96-103 in the 2nd sushi domain.
- Blood.Blood.2014 Jan 9. [Epub ahead of print]
- Coagulation factor XIII (FXIII) is a heterotetramer consisting of two catalytic A subunits (FXIII-A2) and two protective/inhibitory B subunits (FXIII-B2). FXIII-B, a mosaic protein consisting of ten sushi domains, significantly prolongs the lifespan of catalytic subunits in the circulation and preve
- PMID 24408323
Japanese Journal
- Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control
- 臍出血に対し外科的処置を繰り返した先天性XIII因子欠損症の1新生児例
- 臨床室 血液凝固第ⅩⅢ因子の低下による大腿部筋肉内血腫の1例
Related Links
- fibrin-stabilizing factor. See factor XIII. factor [fak´ter] an agent or element that contributes to the production of a result. accelerator factor factor V, one of the coagulation factors. factor I see coagulation factors. factor II see coagulation ...
- fibrin /fi·brin/ (fi´brin) an insoluble protein that is essential to clotting of blood, formed from fibrinogen by action of thrombin. fi·brin (fī′brĭn) n. An elastic, insoluble, whitish protein derived from fibrinogen by the action of thrombin and ...
★リンクテーブル★
[★]
- 英
- factor XIII, F XIII
- 同
- フィブリン安定化因子, fibrin-stabilizing factor, fibrin stabilizing factor, FSF、血漿トランスグルタミナーゼ, plasma transglutaminase、血液凝固第XIII因子、凝固第XIII因子
- 商
- フィブロガミンP
- 関
- 血液凝固因子
- トロンビンにより活性化され、フィブリン線維を架橋してフィブリン網を安定化させる血液凝固因子の一つ。
臨床関連
低下
[★]
- 関
- actual、actually、in fact、in practice、indeed、practically
[★]
- 関
- element、elementary、factorial、parameter