Factor XIII
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| coagulation factor XIII, A1 polypeptide
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| 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
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| Identifiers | |
| Symbol | F13B |
| Entrez | 2165 |
| HUGO | 3534 |
| OMIM | 134580 |
| RefSeq | NM_001994 |
| UniProt | P05160 |
| Other data | |
| Locus | Chr. 1 q31-q32.1 |
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Overview
Factor XIII or fibrin stabilizing factor is an enzyme (EC 2.3.2.13) of the blood coagulation system that crosslinks fibrin. 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.
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
Zymogene factor XIII is a 320000 Mr 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 83000.
- 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 76500.
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
References
- ↑ 1.0 1.1 Laki K, Lorand L. On the solubility of fibrin clots. Science 1948;108:280.
- ↑ 2.0 2.1 2.2 2.3 "Factor XIII: recommended terms and abbreviations.". J Thromb Haemost. PMID 16938124.
External links
Proteins: coagulation | |
|---|---|
| Coagulation factors | intrinsic pathway (FXII, FXI, FIX, FVIII) - extrinsic pathway (Tissue factor, FVII) - common pathway (FX, FV, (Pro)thrombin / FII, Fibrin / FI, FXIII) - HMWK - vWF - Kallikrein |
| Inhibitors | Antithrombin - Protein C - Protein S - Protein Z - ZPI - TFPI |
| Fibrinolysis | Plasmin - tPA/urokinase - PAI-1/2 - α2-AP - α2-macroglobulin - TAFI |
Transferases: acyltransferases (EC 2.3) | |
|---|---|
| 2.3.1: other than amino-acyl groups (mostly acetyltransferases) | N-Acetylglutamate synthase - Choline acetyltransferase - Acetyl-Coenzyme A acetyltransferase - Dihydrolipoyl transacetylase - Acetyl-CoA C-acyltransferase - Beta-galactoside transacetylase - Carnitine O-palmitoyltransferase (CPT1, CPT2) - Acyltransferase like 2 - Chloramphenicol acetyltransferase - Aminolevulinic acid synthase - Beta-ketoacyl-ACP synthase - Glyceronephosphate O-acyltransferase - Lecithin-cholesterol acyltransferase - Histone acetyltransferase (P300/CBP) - Serotonin N-acetyl transferase |
| 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 |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
