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Topic: Factor XIII


In the News (Mon 20 May 13)

  
  NHF | Bleeding Disorders Information Center | Factor XIII Deficiency
Factor XIII is responsible for clot stabilization and cross linking of the fibrin polymer in blood.
Factor XIII deficiency is a severe bleeding disorder usually associated with trauma.
Specific factor XIII assays are needed to confirm a diagnosis of this deficiency.
www.hemophilia.org /bdi/bdi_types11.htm   (544 words)

  
 Factor XIII deficiency - MayoClinic.com   (Site not responding. Last check: 2007-11-06)
Factor XIII deficiency is a rare, inherited bleeding disorder.
Factor XIII is a clotting factor that is important for normal clotting and wound healing.
Factor XIII deficiency is inherited in an autosomal recessive manner.
www.mayoclinic.com /health/factor-xiii-deficiency/AN00508   (268 words)

  
 ZymoGenetics   (Site not responding. Last check: 2007-11-06)
Factor XIII also contributes to the mechanical strength of the clot by attaching the growing clot to the surrounding tissue.
The structure, composition, and activity of the Factor XIII-A subunits from all sources are identical and both forms of the Factor XIII zymogen (plasma [A2B2] and cellular [A2]) are efficiently activated by thrombin.
Factor XIII activity levels in patients with allogeneic haematopoeitic stem cell transplantation and acute graft-versus-host disease of the gut.
www.zgi.com /products/factor-xiii.html   (1295 words)

  
 Haematologic Technologies - Human Factor XIII
Factor XIII is the zymogenic form of the glutaminyl-peptide g-glutamyl transferase factor XIIIa (fibrinoligase, plasma transglutaminase, fibrin stabilizing factor, E.C. Factor XIII is unique among transamidases in that it is a zymogen in vivo (2).
Plasma factor XIII is synthesized in the liver and circulates as a tetramer (Mr=320,000), composed of 2 pairs of nonidentical subunits (A
Factor XIII is purified from fresh frozen human plasma by a modification of the procedures described by Folke (2) and Lorand (10) involving barium citrate, ammonium sulfate and glycine precipitations, ion exchange chromatography and gel filtration.
www.haemtech.com /Zymogens/Factor_XIII.htm   (519 words)

  
 Critical Care | Full text | Effects of coagulation factor XIII on intestinal functional capillary density, leukocyte ...
The objective of this study was to determine the effects of the administration of the coagulation factor XIII (F XIII) on intestinal functional capillary density, leukocyte adherence and mesenteric plasma extravasation during experimental endotoxemia.
F XIII may have multiple effects in addition to its effects in the coagulation system: it plays a role in cell adhesion and migration [5], prevents edema formation due to its influence on endothelial barrier function [6-8], influences constitution of cellular and extracellular matrix [9,10] and it seems to promote wound and bone healing [11-13].
The substitution of F XIII reduced the incidence of myocardial swelling and the authors concluded that the clinical application of F XIII may have a valuable therapeutic benefit in cases of leakage syndrome during extracorporeal circulation in congenital heart surgery [8].
ccforum.com /content/10/1/R29   (4263 words)

  
 FACTOR XIII DEFICIENCY CASE REPORTS PEDIATRIC ONCALL
Factor XIII screening test showed that the clot lysed in less than 1 hour in 5 M urea suggestive of severe Factor XIII deficiency, though he had no previous history of excessive bleeding or bleeding from umbilical cord.
Since Factor XIII is a heterotetramer with 2A chains and 2B protein chains, polymorphism in A and B proteins may lead to clinical manifestation.
Factor XIII is not only in plasma but also in platelets, megakaryocytes and monocytes –; macrophages.
www.pediatriconcall.com /fordoctor/CaseReports/factor.asp   (855 words)

  
 OHC : Factor XIII Deficiency   (Site not responding. Last check: 2007-11-06)
Factor XIII deficiency results in a life-long bleeding tendency in homozygotes, and this is associated with poor wound healing.
Factor XIII is found in plasma as a 320 kD tetramer composed of two 75 kD 'a' subunits linked non-covalently to two 80 kD 'b' subunits.
Factor XIII is activated by thrombin in a calcium-dependent reaction, resulting in dissociation of the 'b' subunits and the cleavage of activation peptides from the active 'a' subunits.
www.medicine.ox.ac.uk /ohc/xiii.htm   (669 words)

  
 Role of factor XIII in fibrin clot formation and effects of genetic polymorphisms -- Ariëns et al. 100 (3): 743 -- ...
Factor XIII and fibrinogen are unusual among clotting factors in that neither is a serine protease.
Plasma factor XIII is a tetrameric molecule composed of 2 A-subunits of 83.2 kd and 2 B-subunits of 79.7 kd that are held
of coronary thrombosis, inhibition of cross-linking by factor
www.bloodjournal.org /cgi/content/full/100/3/743   (6504 words)

  
 Factor XIII
Factor XIII is a large (320 kilodaltons) tetrameric molecule that is composed of two A-chains and two B-chains, A2B2.
Factor XIII is activated by thrombin in the presence of calcium.
The primary function of activated factor XIII (XIIIa) is to catalyze the formation of covalent bonded between fibrin molecules stabilizing the fibrin clot.
www.labcorp.com /datasets/labcorp/html/chapter/mono/cf001300.htm   (640 words)

  
 Factor XIII   (Site not responding. Last check: 2007-11-06)
DNA sequences for binding of myeloid-enriched factors (GATA-1 and Ets-1) were recognized in an upstream region, and the GATA-1 element was found to be responsible for the enhancer activity.
XIII deficient plasma, XIII inhibitor plasma, deficient plasma with replacement therapy were examined for clot solubility by Ca + Urea, Ca + Acetic acid, Ca + MCA, Ca/Thr + Urea, Ca/Thr + Acetic acid, or Ca/Thr + MCA.
Three topics were chosen to be discussed in the next session in Paris; proper screening methods for factor XIII with high sensitivity not to miss its deficiency; reassessment of the assay method for XIII activity regarding thrombin concentration; and collaboration to seek for a standard material for XIII.
www.med.unc.edu /isth/SSC/00sscminutes/00factorxiii.htm   (1002 words)

  
 Homepage for David C. Teller
Factor XIII is a member of a larger family of transglutaminases.
A careful comparison of the structure of factor XIII with Papain (a representative member of the cysteine proteinase family) has revealed that the central core of the enzyme structure as well as the catalytic apparatus is so similar as to indicate divergent evolution from a common ancestral protein.
Factor XIII is a calcium-dependent transglutaminase and covalently crosslinks blood clots in the final stage of the coagulation process.
www.bmsc.washington.edu /people/teller   (709 words)

  
 FACTOR XIII   (Site not responding. Last check: 2007-11-06)
There are 5 common coding polymorphisms in the factor XIII A gene, of which a valine to leucine transition at residue 34 is of interest due to its vicinity to the thrombin cleavage site and its relation to thrombotic disorders.
He showed that early covalent cross-linking of the fibrin clot by factor XIII Leu34 reduced lateral aggregation of the fibrin fibers, leading to a reduction in fiber thickness from 121.0 +/- 23.9 nm to 75.7 +/- 11.3 nm and alteration in rates of fibrinolysis of the fibrin clot.
Factor XIII, one of the active ingredients of the fibrin sealant, "Bolheal(R)," is included in the fibrinogen component.
www.med.unc.edu /isth/SSC/01sscminutes/01factorxiii.html   (1818 words)

  
 News Release
Factor XIII is the terminal enzyme in the coagulation cascade.
Factor XIII enhances clot strength by crosslinking fibrin strands within the clot and increases resistance of the clot to fibrinolysis by crosslinking enzyme inhibitors, such as alpha 2 plasmin inhibitor, into the clot.
In addition, rFactor XIII was observed to have a long half-life (9-13 days), an important finding that may allow for extended dosing schedules for the delivery of the protein.
www.zymogenetics.com /ir/newsItem.php?id=477230   (1453 words)

  
 Prenatal diagnosis in factor XIII-A deficiency -- Killick et al. 80 (3): 238 -- Archives of Disease in Childhood - ...
Congenital factor XIII deficiency is a severe bleeding disorder that is inherited as an autosomal recessive trait.
Congenital factor XIII deficiency is a rare inherited bleeding disorder that commonly occurs in neonates.
Factor XIII circulates in plasma as a heterotetramer of two catalytic A subunits and two B subunits.
fn.bmjjournals.com /cgi/content/full/80/3/F238   (1421 words)

  
 Human blood coagulation factor XIII   (Site not responding. Last check: 2007-11-06)
Activated factor XIII is the prototypical enzyme of a family of transglutaminases and related proteins found universally among animals.
Factor XIII is activated to factor XIIIa by thrombin in the final step of the coagulation cascade.
Factor XIIIa is a transglutaminase serving to cross-link fibrin to form a stable clot.
www.bmsc.washington.edu /projects/fxiii.html   (366 words)

  
 Genomics|HuGENet|Reviews|VT and Factor XIII Val34Leu|PubMed ID: 16740590
Factor XIII (FXIII), also called fibrin stabilizing factor, has a crucial role in the blood coagulation and fibrinolytic pathways.
Plasma FXIII is an inactive enzyme precursor that circulates in plasma in the form of two pairs of nonidentical A and B subunits.
Thrombophilia is a highly prevalent problem, with more than 10 percent of the population affected by one of the currently known genetic risk factors, the common ones being factor V Leiden and Prothrombin G20210A, and it is identified in at least 50 percent of cases of VTE (21).
www.cdc.gov /genomics/hugenet/reviews/VTfactorXIII.htm   (3873 words)

  
 Basic Science - Thrombosis and Transglutaminase Research Laboratory - Duke Comprehensive Cancer Center   (Site not responding. Last check: 2007-11-06)
Factor XIII once activated by thrombin is responsible for catalyzing the covalent coupling of fibrin molecules to generate a blood clot that is resistant to mechanical and proteolytic disruption.
Cleavage of blood coagulation factor XIII and fibrinogen by thrombin during in vitro clotting.
Factor XIII has a very low affinity for calcium and several sites were reported as calcium binding domains.
www.cancer.duke.edu /ttrl/basicscience.asp   (5518 words)

  
 Hemophilia Galaxy | Factor Deficiencies
The complete absence of Factor I, or afibrinogenemia, usually is discovered in newborns, due to bleeding from the umbilical cord, urinary tract, or central nervous system.
This rare disorder involves a deficiency of Factor XIII, also known as fibrin stabilization factor, which is necessary for proper clot formation and wound healing.
This disorder is one of the rarer of the inherited factor deficiencies, affecting an estimated one in three million people.
www.hemophiliagalaxy.com /patients/about/rarebleed/factordef.html   (844 words)

  
 Factor XIII - Wikipedia, the free encyclopedia
Factor XIII or fibrin stabilizing factor is an enzyme (EC 2.3.2.13) of the blood coagulation system that crosslinks fibrin.
Factor XIII is activated by thrombin into factor XIIIa; its activation into Factor XIIIa requires calcium as a cofactor.
A clot that has not been stabilized by FXIIIa is soluble in 5 M urea, while a stabilized clot is resistant to this phenomenon.
en.wikipedia.org /wiki/Factor_XIII   (398 words)

  
 Factor XIII Deficiency : BPL (Bio Products Laboratory)
Factor XIII deficiency is a very rare bleeding disorder, affecting 1 in 5 million men and women in the UK.
Factor XIII is also known as Fibrin Stabilising Factor (FSF) and the deficiency can be both inherited and acquired.
Replacement therapy in Factor XIII deficiency has proved to be generally straightforward, owing to the low levels of Factor XIII required to control bleeding.
www.bpl.co.uk /public/therapy_areas/coagulation/factor_xiii.asp   (144 words)

  
 Population Pharmacokinetics of Recombinant Factor XIII in Cynomolgus Monkeys
Population Pharmacokinetics of Recombinant Factor XIII in Cynomolgus Monkeys
Factor XIII (FXIII) stabilizes clots primarily by cross-linking fibrin, thus protecting a newly formed clot from fibrinolysis by plasmin.
6. Wolpl A, Lattke H, Board PG, et al.  Coagulation factor XIII A and B subunits in bone marrow and liver transplantation. Transplantation. 1987;43:151-153.
www.aapspharmsci.org /view.asp?art=aapsj070370   (5320 words)

  
 Hemostasis Reference Laboratory | Factor XIII Screen (by Urea Solubility)
Factor XIII forms covalent cross links between fibrin chains.
In the absence of Factor XIII, the fibrin clot will be dissolved by 5 M urea which disrupts the hydrogen bonds.
Hereditary factor XIII deficiency, an autosomal recessive disorder, should be suspected in a patient with delayed post-surgical or traumatic bleeding, unexplained CNS hemorrhage, poor wound healing, or recurrent abortions.
www.psbc.org /lab_hemostasis/test03.htm   (96 words)

  
 Factor XIII Deficiency
It is possible that the main title of the report Factor XIII Deficiency is not the name you expected.
Factor XIII Deficiency is an extremely rare inherited blood disorder characterized by abnormal blood clotting that may result in abnormal bleeding.
Factor XIII Deficiency may be inherited as an autosomal dominant genetic trait.
www.webmd.com /hw/raising_a_family/nord66.asp   (437 words)

  
 Factor XIII Val34Leu Variant Is Protective against Venous Thromboembolism: A HuGE Review and Meta-Analysis -- Wells et ...
Localization of the coagulation factor XIII B subunit gene (F13B) to chromosome bands 1q31-32.1 and restriction fragment length polymorphism at the locus.
Ethnic heterogeneity of the factor XIII Val34Leu polymorphism.
Factor XIII Val34Leu is a genetic factor involved in the aetiology of venous thrombosis.
aje.oxfordjournals.org /cgi/content/full/164/2/101   (4217 words)

  
 Information Centre for Rare Diseases and Orphan Drugs :: News
Hereditary factor XIII deficiency is a rare bleeding disorder mostly due to mutations in FXIII A subunit.
Factor XIII (FXIII) is a plasma transglutaminase circulating in blood as a heterotetramer and consisting of two catalytic A subunits and two non-catalytic B subunits.
Coagulation factor XIII is a transglutaminase that catalyzes crosslink formation in fibrin clots.
www.raredis.org /modules/news/article.php?storyid=277   (188 words)

  
 Fibronectin - Molecular Interactions - factor XIII
Factor XIII is a member of the transglutaminase family of cross-linking enzymes that form covalent isopeptide bonds between the side chains of specific Gln and Lys residues on certain proteins [ref].
When factor XIIIa is added to fibroblasts growing in culture it co-localizes with extracellular fibronectin-containing fibrils where it remains active and cross-links fibronectin molecules to each other but not to the collagen in those cultures [ref].
The b subunits of factor XIII contain an Arg-Gly-Asp sequence which could interact with integrins on the cell surface.
home.comcast.net /~kennethingham/newsite/coag/fxiii.htm   (810 words)

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