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F13A1 gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

F13A1 gene

The F13A1 gene encodes the A subunit of coagulation factor XIII, a protein that stabilizes blood clots and supports wound healing.

Overview

The F13A1 gene provides instructions for making one part, the A subunit, of a protein called factor XIII. This protein is part of a group of related proteins called coagulation factors that are essential for normal blood clotting. They work together as part of the coagulation cascade, which is a series of chemical reactions that forms blood clots in response to injury. After an injury, clots seal off blood vessels to stop bleeding and trigger blood‑vessel repair. Factor XIII acts at the end of the cascade to strengthen and stabilize newly formed clots, preventing further blood loss.

Factor XIII in the bloodstream is made of two A subunits (produced from the F13A1 gene) and two B subunits (produced from the F13B gene). When a new blood clot forms, the A and B subunits separate from one another, and the A subunit is cut (cleaved) to produce the active form of factor XIII (factor XIIIa). The active protein links together molecules of fibrin, the material that forms the clot, which strengthens the clot and keeps other molecules from breaking it down.

Studies suggest that factor XIII has additional functions, although these are less well understood than its role in blood clotting. Specifically, factor XIII is likely involved in other aspects of wound healing, immune system function, maintaining pregnancy, bone formation, and the growth of new blood vessels (angiogenesis).

Health Conditions Related to Genetic Changes

Factor XIII deficiency

At least 140 mutations in the F13A1 gene have been found to cause inherited factor XIII deficiency, a rare bleeding disorder. Without treatment, affected individuals have a greatly increased risk of abnormal bleeding episodes, including life‑threatening bleeding inside the skull (intracranial hemorrhage). F13A1 gene mutations severely reduce the amount or activity of the A subunit of factor XIII. In most people with these mutations, the level of functional factor XIII in the bloodstream is less than 5 percent of normal. This loss of factor XIII activity weakens new blood clots and prevents them from stopping blood loss effectively.

Other disorders

Several common variations (polymorphisms) in the F13A1 gene have been studied as possible risk factors for diseases involving abnormal blood clotting. The most common F13A1 gene polymorphism changes a single protein building block (amino acid) in a critical part of the A subunit, replacing the amino acid valine with the amino acid leucine at protein position 34 (written as Val34Leu or V34L). This genetic change speeds up the activation of factor XIII. The Val34Leu polymorphism has been studied in relation to heart disease, stroke, recurrent pregnancy loss, and several other conditions. However, the results of these studies have been conflicting, and it remains unclear whether the polymorphism represents a major risk factor for any of these conditions.

Other Names for This Gene

  • bA525O21.1 (coagulation factor XIII, A1 polypeptide)
  • coagulation factor XIII A chain precursor
  • coagulation factor XIII, A polypeptide
  • coagulation factor XIII, A1 polypeptide
  • coagulation factor XIIIa
  • F13A
  • factor XIIIa
  • fibrin stabilizing factor, A subunit
  • fibrinoligase
  • FSF, A subunit
  • protein-glutamine gamma-glutamyltransferase A chain
  • TGase
  • transglutaminase A chain
  • transglutaminase. plasma

Additional Information & Resources

Tests Listed in the Genetic Testing Registry

  • Tests of F13A1

Scientific Articles on PubMed

  • PubMed

Catalog of Genes and Diseases from OMIM

  • FACTOR XIII, A1 SUBUNIT; F13A1

Gene and Variant Databases

  • NCBI Gene
  • ClinVar

References

  • Bagoly Z, Koncz Z, Harsfalvi J, Muszbek L. Factor XIII, clot structure, thrombosis. Thromb Res. 2012 Mar;129(3):382-7. doi: 10.1016/j.thromres.2011.11.040. Epub 2011 Dec 24. Citation on PubMed
  • Biswas A, Ivaskevicius V, Seitz R, Thomas A, Oldenburg J. An update of the mutation profile of Factor 13 A and B genes. Blood Rev. 2011 Sep;25(5):193-204. doi: 10.1016/j.blre.2011.03.001. Epub 2011 Jun 2. Citation on PubMed
  • Biswas A, Ivaskevicius V, Thomas A, Oldenburg J. Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. Hamostaseologie. 2014;34(2):160-6. doi: 10.5482/HAMO-13-08-0046. Epub 2014 Feb 7. Citation on PubMed
  • Hethershaw EL, Cilia La Corte AL, Duval C, Ali M, Grant PJ, Ariens RA, Philippou H. The effect of blood coagulation factor XIII on fibrin clot structure and fibrinolysis. J Thromb Haemost. 2014 Feb;12(2):197-205. doi: 10.1111/jth.12455. Citation on PubMed
  • Levy JH, Greenberg C. Biology of Factor XIII and clinical manifestations of Factor XIII deficiency. Transfusion. 2013 May;53(5):1120-31. doi: 10.1111/j.1537-2995.2012.03865.x. Epub 2012 Aug 28. Citation on PubMed
  • Muszbek L, Bereczky Z, Bagoly Z, Komaromi I, Katona E. Factor XIII: a coagulation factor with multiple plasmatic and cellular functions. Physiol Rev. 2011 Jul;91(3):931-72. doi: 10.1152/physrev.00016.2010. Citation on PubMed
  • Schroeder V, Kohler HP. Factor XIII deficiency: an update. Semin Thromb Hemost. 2013 Sep;39(6):632-41. doi: 10.1055/s-0033-1353392. Epub 2013 Aug 8. Citation on PubMed

Genomic Location

The F13A1 gene is found on chromosome 6.

Frequently Asked Questions

The mainstay of treatment is intravenous infusion of purified factor XIII concentrate, which restores clot stability and reduces bleeding episodes. In some cases, cryoprecipitate or fresh frozen plasma can be used, but they contain lower levels of factor XIII and are less effective. Long‑term management may involve regular prophylactic infusions, especially before surgeries or dental procedures, to maintain adequate factor XIII activity.
Patients with inherited factor XIII deficiency often present with delayed wound healing, easy bruising, and prolonged bleeding after surgery or injury. They may also experience spontaneous bleeding in joints or the gastrointestinal tract. Because the clot is initially formed but not stabilized, bleeding can be subtle and may not be detected until a significant injury occurs. Early recognition usually involves a detailed bleeding history and a coagulation profile that shows normal PT, aPTT, and fibrinogen but a low factor XIII activity level.
Factor XIII plays a role in placental development and maintaining the integrity of the uterine lining. Women with factor XIII deficiency are at increased risk for miscarriage, preterm delivery, and postpartum hemorrhage. Prenatal screening for factor XIII activity and close monitoring during delivery can reduce complications. Replacement therapy with factor XIII concentrate before and after delivery is often recommended to prevent excessive bleeding.
While there is no specific diet that increases factor XIII levels, maintaining a healthy weight, avoiding alcohol, and ensuring adequate vitamin K intake can support overall coagulation function. Patients should also avoid medications that inhibit platelet function (e.g., aspirin, NSAIDs) unless prescribed by a hematologist, as these can exacerbate bleeding risk. Regular exercise and avoiding high‑impact sports can reduce injury risk in individuals with factor XIII deficiency.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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