Skip to main content
NCBI home page
The Journal of Clinical Investigation logoLink to The Journal of Clinical Investigation
. 1973 Oct;52(10):2398–2403. doi: 10.1172/JCI107429

Immunological Studies of Coagulation Factor XIII

E D Israels 1,2, F Paraskevas 1,2, L G Israels 1,2
PMCID: PMC302497  PMID: 4199604

Abstract

Human fibrin-stabilizing factor (Factor XIII) has been studied immunologically by the preparation of specific anti-Factor XIII antiserum in rabbits. On immunodiffusion it was found that normal plasma produced two precipitin lines. One of the precipitin lines was identical with that present in soluble platelet extract (the α-component), the other with that present in normal serum (β-component). Plasma and serum of patients with congenital Factor XIII deficiency contained only the β-component. By adsorption it was possible to prepare a second antiserum with solely anti-α-activity that did not react with the serum or plasma of XIII-deficient patients. Both antisera neutralized the clot-stabilizing activity of normal plasma. The action of thrombin on fibrinogen-free plasma or platelet extract abolished the immunoprecipitin α-line but did not reduce the capacity to neutralize antibody as measured by clot stabilization.

It is concluded that the plasma Factor XIII molecule consists of two immunologically identifiable components, α and β. The clot-stabilizing activity and thrombin-reactive site are situated on the α-component. Patients with congenital Factor XIII deficiency are devoid of immunologically identifiable or functional α-component but retain immunologically identifiable β-component. It is this β-component that accounts for the observed immunologically detectable Factor XIII in those patients devoid of clot-stabilizing activity.

Full text

PDF
2398

Images in this article

2399Image
on p.2399
2399Image
on p.2399
2400Image
on p.2400

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Avrameas S., Ternynck T. Biologically active water-insoluble protein polymers. I. Their use for isolation of antigens and antibodies. J Biol Chem. 1967 Apr 10;242(7):1651–1659. [PubMed] [Google Scholar]
  2. Bohn H., Haupt H., Kranz T. Die molekulare Struktur der fibrinstabilisierteenden Faktoren des Menschen. Blut. 1972 Oct;25(4):235–248. doi: 10.1007/BF01632188. [DOI] [PubMed] [Google Scholar]
  3. Bohn H. Immunochemical studies on the fibrin stabilizing factors from human plasma and platelets. Blut. 1971 May;22(5):237–243. doi: 10.1007/BF01678915. [DOI] [PubMed] [Google Scholar]
  4. DAVIS B. J. DISC ELECTROPHORESIS. II. METHOD AND APPLICATION TO HUMAN SERUM PROTEINS. Ann N Y Acad Sci. 1964 Dec 28;121:404–427. doi: 10.1111/j.1749-6632.1964.tb14213.x. [DOI] [PubMed] [Google Scholar]
  5. Duckert F. Hereditärer Mangel an Factor-XIII-Aktivität-synthese eines funktionslosen Proteins. Schweiz Med Wochenschr. 1970 Feb 14;100(7):328–328. [PubMed] [Google Scholar]
  6. Egbring R., Andrassy K., Egli H., Meyer-Lindenberg J. Untersuchungen bei zwei Patienten mit kongenitalem Faktor-XII-Mangel. (Zugleich ein Beitrag zur Problematik der Bestimmung des Faktors XIII) Thromb Diath Haemorrh. 1970 May 31;23(2):313–339. [PubMed] [Google Scholar]
  7. Harding H. W., Rogers G. E. Formation of the -( -glutamyl) lysine cross-link in hair proteins. Investigation of transamidases in hair follicles. Biochemistry. 1972 Jul 18;11(15):2858–2863. doi: 10.1021/bi00765a019. [DOI] [PubMed] [Google Scholar]
  8. Hartman B. K., Udenfriend S. A method for immediate visualization of proteins in acrylamide gels and its use for preparation of antibodies to enzymes. Anal Biochem. 1969 Sep;30(3):391–394. doi: 10.1016/0003-2697(69)90132-8. [DOI] [PubMed] [Google Scholar]
  9. Kiesselbach T. H., Wagner R. H. Fibrin-stabilizing factor: a thrombin-labile platelet protein. Am J Physiol. 1966 Dec;211(6):1472–1476. doi: 10.1152/ajplegacy.1966.211.6.1472. [DOI] [PubMed] [Google Scholar]
  10. LOEWY A. G., DUNATHAN K., KRIEL R., WOLFINGER H. L., Jr Fibrinase. I. Purification of substrate and enzyme. J Biol Chem. 1961 Oct;236:2625–2633. [PubMed] [Google Scholar]
  11. McDonagh J., McDonagh R. P., Jr, Delâge J. M., Wagner R. H. Factor XIII in human plasma and platelets. J Clin Invest. 1969 May;48(5):940–946. doi: 10.1172/JCI106053. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. PROCTOR R. R., RAPAPORT S. I. The partial thromboplastin time with kaolin. A simple screening test for first stage plasma clotting factor deficiencies. Am J Clin Pathol. 1961 Sep;36:212–219. doi: 10.1093/ajcp/36.3.212. [DOI] [PubMed] [Google Scholar]
  13. Schwartz M. L., Pizzo S. V., Hill R. L., McKee P. A. The subunit structures of human plasma and platelet factor XIII (fibrin-stabilizing factor). J Biol Chem. 1971 Sep 25;246(18):5851–5854. [PubMed] [Google Scholar]
  14. Takagi T., Konishi K. Purification and some properties of fibrin stabilizing factor. Biochim Biophys Acta. 1972 Jul 21;271(2):363–370. doi: 10.1016/0005-2795(72)90211-5. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Clinical Investigation are provided here courtesy of American Society for Clinical Investigation

RESOURCES