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Encyclopedia > Factor XIII

Factor XIII or fibrin stabilizing factor is an enzyme (EC 2.3.2.13 (http://us.expasy.org/cgi-bin/nicezyme.pl?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.

Contents

Enzyme

Factor XIII crosslinks fibrin
Factor XIII crosslinks fibrin

Factor XIII consists 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.
  • B subunit (1q31-q32.1). This has no clear enzymatic activity, and may serve as a carrier for the A subunit.

Role in disease

Factor XIII deficiency may occur very rarely, and can cause a severe bleeding tendency.


See also

External links

  • OMIM 134570 (http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134570) (A subunit) and OMIM 134580 (http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=134580) (B subunit)
  • Factor XIII deficiency (http://www.hemophilia.org/bdi/bdi_types11.htm)


Cardiovascular system - Blood
Red blood cells - White blood cells - Platelets - Blood plasma
White blood cells
Granulocytes (Neutrophil granulocytes, Eosinophil granulocytes, Basophil granulocytes) - Lymphocytes - Monocytes
Coagulation
Coagulation factors: - Fibrin - Thrombin - FVII - FVIII - FIX - FXII - FXIII - HMWK - vWF
Inhibitors: Antithrombin - Protein C - Protein S - TFPI
Fibrinolysis: Plasmin - tPA/uPA - PAI-1/2 - α2-AP

  Results from FactBites:
 
NHF | Bleeding Disorders Information Center | Factor XIII Deficiency (544 words)
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.
  More results at FactBites »

 
 

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