Coagulation factor XIII, A1 polypeptide

Jump to: navigation, search
VALUE_ERROR (nil)
Identifiers
Aliases
External IDsGeneCards: [1]
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

n/a

n/a

RefSeq (protein)

n/a

n/a

Location (UCSC)n/an/a
PubMed searchn/an/a
Wikidata
View/Edit Human

Coagulation factor XIII A chain is a protein that in humans is encoded by the F13A1 gene.

Function

This gene encodes the coagulation factor XIII A subunit. Coagulation factor XIII is the last zymogen to become activated in the blood coagulation cascade. Plasma factor XIII is a heterotetramer composed of 2 A subunits and 2 B subunits. The A subunits have catalytic function, and the B subunits do not have enzymatic activity and may serve as plasma carrier molecules. Platelet factor XIII is composed of just 2 A subunits, which are identical to those of plasma origin. Upon cleavage of the activation peptide by thrombin and in the presence of calcium ion, the plasma factor XIII dissociates its B subunits and yields the same active enzyme, factor XIIIa, as platelet factor XIII. This enzyme acts as a transglutaminase to catalyze the formation of gamma-glutamyl-epsilon-lysine crosslinking between fibrin molecules, thus stabilizing the fibrin clot. It also crosslinks alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin. Factor XIII deficiency is classified into two categories: type I deficiency, characterized by the lack of both the A and B subunits; and type II deficiency, characterized by the lack of the A subunit alone. These defects can result in a lifelong bleeding tendency, defective wound healing, and habitual abortion.[1]

Interactions

Coagulation factor XIII, A1 polypeptide has been shown to interact with F13B.[2][3]

References

  1. "Entrez Gene: F13A1 coagulation factor XIII, A1 polypeptide".
  2. Carrell NA, Erickson HP, McDonagh J (Jan 1989). "Electron microscopy and hydrodynamic properties of factor XIII subunits". J. Biol. Chem. 264 (1): 551–6. PMID 2491853.
  3. Achyuthan KE, Rowland TC, Birckbichler PJ, Lee KN, Bishop PD, Achyuthan AM (Sep 1996). "Hierarchies in the binding of human factor XIII, factor XIIIa, and endothelial cell transglutaminase to human plasma fibrinogen, fibrin, and fibronectin". Mol. Cell. Biochem. 162 (1): 43–9. doi:10.1007/bf00250994. PMID 8905624.

Further reading



Linked-in.jpg