Fibrin cross-linking in congenital factor XIII deficiency.

Abstract

Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. Quantitative studies of transamidase activity of the factor are available in only a few cases, and the fibrin cross-linking pattern is not well known. The present paper deals with the quantitative estimation of factor XIII transamidase activity (dansylcadaverine system), factor XIII molecular subunits, and the corresponding fibrin cross-linking pattern in seven homozygous patients with factor XIII deficiency. The results indicate that transamidase activity was present in all patients, and the range was 0.5-1.7%. The pattern of fibrin stabiisation showed an absence of cross-linking in two patients, the presence of gamma-gamma-dimers (traces) in four, and gamma-gamma-dimers plus incomplete alpha-polymers (traces) in one patient. In conclusion, the homozygous patients reported here were not completely devoid of functioning factor XIII.

• Authors: F Rodeghiero, T Barbui
• Journal: Journal of clinical pathology (J Clin Pathol) Vol. 33 Issue 5 Pg. 434-7 (May 1980) ISSN: 0021-9746 [Print] England
• PMID: 7400341 (Publication Type: Journal Article)

Chemical References

• Fibrin
• Factor XIII
• Thrombin

Topics

• Electrophoresis, Polyacrylamide Gel
• Enzyme Activation
• Factor XIII (analysis, metabolism)
• Factor XIII Deficiency (blood)
• Fibrin
• Humans
• Protein Binding
• Thrombin