Fibrin cross-linking in congenital factor XIII deficiency.
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.
D004591:Electrophoresis, Polyacrylamide Gel; D004789:Enzyme Activation; D005176:Factor XIII / Q000032:analysis / Q000378:metabolism; D005177:Factor XIII Deficiency / Q000097:blood*; D005337:Fibrin; D006801:Humans; D011485:Protein Binding; D013917:Thrombin
D005337:Fibrin; D005176:Factor XIII; D013917:Thrombin