第XIII因子。フィブリン安定化因子 fibrin stabilizing factor
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"
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
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/08/30 23:57:45」(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
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.
English Journal
- Protein predictive signatures for lymph node metastasis of gastric cancer.
- Li W, Ye F, Wang D, Sun X, Tong W, Lian G, Jiang J, Suo J, Zhang DY.SourceDepartment of General Surgery, The First Hospital, Jilin University, Changchun, Jilin, China.
- International journal of cancer. Journal international du cancer.Int J Cancer.2013 Apr 15;132(8):1851-9. doi: 10.1002/ijc.27864. Epub 2012 Oct 25.
- Lymph node status remains one of most crucial indicators of gastric cancer prognosis and treatment planning. Current imaging methods have limited accuracy in predicting lymph node metastasis. We sought to identify protein markers in primary gastric cancer and to define a risk model to predict lymph
- PMID 23011604
- The effect of whole-body cooling on hematological and coagulation parameters in asphyxic newborns.
- Oncel MY, Erdeve O, Calisici E, Oguz SS, Canpolat FE, Uras N, Dilmen U.SourceDivision of Neonatology, Zekai Tahir Burak Maternity Teaching Hospital , Ankara , Turkey.
- Pediatric hematology and oncology.Pediatr Hematol Oncol.2013 Apr;30(3):246-52. doi: 10.3109/08880018.2013.771240. Epub 2013 Feb 27.
- Although moderate therapeutic hypothermia is the only proven neuroprotective therapy in neonatal hypoxic ischemic encephalopathy secondary to perinatal asphyxia (PA), there is lack of data for its effect on hemostasis. To investigate the effect of neonatal asphyxia on hemostasis and to evaluate the
- PMID 23445486
- Factor XIII improves platelet adhesion to fibrinogen by protein disulfide isomerase-mediated activity.
- Lahav J, Tvito A, Bagoly Z, Dardik R, Inbal A.SourceHemostasis Laboratory, Rabin Medical Center, Beilinson Hospital, Petach Tikva and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
- Thrombosis research.Thromb Res.2013 Apr;131(4):338-41. doi: 10.1016/j.thromres.2012.12.003. Epub 2012 Dec 28.
- BACKGROUND: Factor XIII (FXIII), a plasma pro-transglutaminase, consists of two A subunits and two B subunits (FXIIIA2B2). Following activation by thrombin, it cross-links fibrin chains at the final step of coagulation. We previously reported that FXIII subunit A (FXIIIA) serves as a protein disulfi
- PMID 23276530
Japanese Journal
- 新規D-dimer試薬LPIAジェネシスDダイマーの基礎性能評価および試薬特性
- 下仮屋 雄二,渡邊 真希,坂口 茜,西川 美有,秋月 基伸,桜井 錠治,森本 誠,中谷 中
- 医学検査 68(1), 85-91, 2019
- … 凝固第XIII因子欠乏血漿を凝固させた後に線溶させた結果,経過時間に伴う測定値の上昇を認めるがACE-DDより低値であった。 …
- NAID 130007557519
- New Biomarkers for Prediction of Disseminated Intravascular Coagulation in Patients With Sepsis
- Wakabayashi Ichiro,Mambo Naomi,Ueda Takahiro,Nonaka Daisuke,Lee Lyang-Ja,Tanaka Kenji,Kotani Joji
- Clinical and Applied Thrombosis/Hemostasis 24(9S), 223S-229S, 2018-12-01
- … The peptides were fragments of serum amyloid A-2 protein, α2-HS-glycoprotein, fibrinogen α chain, fibrinogen β chain, serum albumin, collagen α1 (I) chain, collagen α1 (III) chain, and coagulation factor XIII A chain. …
- NAID 120006550268
- 血液凝固第XIII因子製剤投与が有効であった胃切除術後難治性乳糜腹水の1例
- 砂川 祐輝,鹿野 敏雄,寺本 仁,丸山 浩高,蜂須賀 丈博
- 日本臨床外科学会雑誌 79(7), 1453-1458, 2018
- … なかった.血液凝固第XIII因子活性が62%と低値であったため,術後24~28日目に血液凝固第XIII因子製剤を投与した.術後31日目に食事を再開したが,ドレーン排液の白濁化は認めず,その後も腹水増加を疑う所見を認めず,乳糜腹水は改善した.<BR>乳糜腹水は腹部手術後の合併症の一つで,その治療に難渋することがある.乳糜腹水に対し血液凝固第XIII因子製剤を投与した報 …
- NAID 130007585159
Related Links
- Information on Factor XIII (Factor 13) Deficiency ... Welcome Welcome to the first (and only) site in the world that provides information exclusively on Factor XIII (Factor 13). If you are one of the people having Factor XIII deficiency, you ...
- Factor XIII (FXIII), which was initially termed fibrin stabilizing factor, is involved in clot preservation. FXIII deficiency, an autosomal recessive disorder, is a rare but potentially life-threatening cause of a hemorrhagic diathesis. Paradoxically, alterations in FXIII may also predispose to ...
Related Pictures
★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
[★]
第XIII因子, factor XIII。フィブリン安定化因子
[★]
- 関
- coagulation factor XIII、factor XIII
[★]
- 関
- blood coagulation factor XIII、factor XIII
[★]
活性化第XIII因子
- 関
- activated factor XIII
[★]
- 関
- actual、actually、in fact、in practice、indeed、practically
[★]
- 関
- element、elementary、factorial、parameter