Factor xiii

Factor XIII or Fibrin Stabilizing Factor is an enzyme that plays a crucial role in the blood clotting process. It is responsible for the final step of the coagulation cascade, which is the cross-linking of fibrin. This cross-linking strengthens the fibrin clot and helps to prevent premature clot breakdown (fibrinolysis).

Structure

Factor XIII is a transglutaminase that circulates in the blood as a tetramer (two A subunits and two B subunits). The A subunits carry the catalytic site, while the B subunits have a regulatory role and protect the A subunits from premature activation.

Function

The primary function of Factor XIII is to stabilize the fibrin clot. Once a clot has formed, Factor XIII is activated by thrombin, and it then cross-links the fibrin strands, making the clot more stable and resistant to fibrinolysis. This is an important step in the hemostasis process, as it ensures that the clot remains in place until the damaged vessel has had time to heal.

Clinical significance

Deficiency in Factor XIII can lead to a rare bleeding disorder known as Factor XIII deficiency. This condition is characterized by poor clot stability and a tendency to bleed excessively. It can be diagnosed through a clot solubility test and is treated with Factor XIII concentrate.

Factor XIII deficiency can be either inherited or acquired. Inherited Factor XIII deficiency is a rare genetic disorder, while acquired Factor XIII deficiency can occur as a result of liver disease, inflammatory bowel disease, or certain types of cancer.

See also

• Coagulation
• Fibrin
• Thrombin
• Hemostasis
• Factor XIII deficiency

References

Factor xiii Resources
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