Factor XIII


Factor XIII

protein
Name = coagulation factor XIII, A1 polypeptide
caption =


width =
HGNCid = 3531
Symbol = F13A1
AltSymbols = F13A
EntrezGene = 2162
OMIM = 134570
RefSeq = NM_000129
UniProt = P00488
PDB =
ECnumber =
Chromosome = 6
Arm = p
Band = 24
LocusSupplementaryData = .2-p23
protein
Name = coagulation factor XIII, B polypeptide
caption =


width =
HGNCid = 3534
Symbol = F13B
AltSymbols =
EntrezGene = 2165
OMIM = 134580
RefSeq = NM_001994
UniProt = P05160
PDB =
ECnumber =
Chromosome = 1
Arm = q
Band = 31
LocusSupplementaryData = -q32.1

Factor XIII or fibrin stabilizing factor is an enzyme (EC number|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.Laki K, Lorand L. On the solubility of fibrin clots. "Science" 1948;108:280.] A 2005 conference recommended standardization of nomenclature.cite journal | author = Muszbek, L.| title = Factor XIII: recommended terms and abbreviations | journal = J Thromb Haemost | volume = 5| issue = | pages = 181| year = 2007| pmid = 16938124| doi = 10.1111/j.1538-7836.2006.02182.x]

Genetics

Zymogene factor XIII is a 320000 Mr glycoprotein tetramer consisting of twice two subunits (2 A and 2 B), 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.

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.

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.

ee also

* Factor XIII deficiency
* D-dimer

References

External links

* [http://www.hemophilia.org/bdi/bdi_types11.htm Factor XIII deficiency at hemophilia.org]
*


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Look at other dictionaries:

  • factor XIII — Factor de la coagulación presente en el plasma normal que actúa junto con el calcio para producir el coágulo de fibrina insoluble. Diccionario Mosby Medicina, Enfermería y Ciencias de la Salud, Ediciones Hancourt, S.A. 1999 …   Diccionario médico

  • factor XIII — thər(t) tēn, thər(t) n a clotting factor that causes monomeric fibrin to polymerize and become stable and insoluble called also fibrinase see TRANSGLUTAMINASE * * * see under coagulation f s …   Medical dictionary

  • factor XIII — noun in the clotting of blood thrombin catalyzes factor XIII into its active form (fibrinase) which causes fibrin to form a stable clot • Syn: ↑fibrinase • Hypernyms: ↑coagulation factor, ↑clotting factor …   Useful english dictionary

  • Factor XIII — Factor estabilizador de la fibrina (también FSF, Factor Laki Lorand, Fibrinasa, Transagluminasa plasmática o Fibrinoligasa) …   Diccionario de siglas médicas y otras abreviaturas

  • Factor XIII — Fibrinstabilisierender Faktor Schema der Vernetzung durch Faktor XIII …   Deutsch Wikipedia

  • Factor XIII deficiency — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 31412 ICD10 = ICD10|D|68|2|d|65 ICD9 = ICD9|286.3 ICDO = OMIM = 134570 OMIM mult = OMIM2|134580 | MedlinePlus = eMedicineSubj = ped eMedicineTopic = 3040 MeshID = D005177 Factor XIII… …   Wikipedia

  • Coagulation factor XIII, A1 polypeptide — PDB rendering based on 1evu …   Wikipedia

  • Factor de coagulación — Saltar a navegación, búsqueda Los factores de coagulación son todas aquellas proteínas originales de la sangre que participan y forman parte del coágulo sanguíneo. Son trece los factores de coagulación, nombrados con números romanos, todos ellos… …   Wikipedia Español

  • factor de coagulación — m. hemat. Cada una de las 13 sustancias necesarias para la formación de la fibrina y cuya acción conjunta es la responsable del proceso de coagulación de la sangre. Medical Dictionary. 2011. factor de coagulación …   Diccionario médico

  • factor Laki-Lorand — Véase factor XIII. Diccionario Mosby Medicina, Enfermería y Ciencias de la Salud, Ediciones Hancourt, S.A. 1999 …   Diccionario médico