Coagulation factor XIII A chain

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

F13A1
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesF13A1, F13A, coagulation factor XIII A chain
External IDsOMIM: 134570; MGI: 1921395; HomoloGene: 20077; GeneCards: F13A1; OMA:F13A1 - orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_000129

NM_001166391
NM_028784

RefSeq (protein)

NP_000120

NP_001159863
NP_083060

Location (UCSC)Chr 6: 6.14 – 6.32 MbChr 13: 37.05 – 37.23 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Function

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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.[5]

Interactions

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Coagulation factor XIII A chain has been shown to interact with coagulation factor XIII B chain.[6][7]

References

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  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000124491Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000039109Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ "Entrez Gene: F13A1 coagulation factor XIII, A1 polypeptide".
  6. ^ Carrell NA, Erickson HP, McDonagh J (Jan 1989). "Electron microscopy and hydrodynamic properties of factor XIII subunits". J. Biol. Chem. 264 (1): 551–6. doi:10.1016/S0021-9258(17)31294-2. PMID 2491853.
  7. ^ 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. S2CID 23583301.

Further reading

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