Biomaterial Database

Fibrin cross-linking in congenital factor XIII deficiency. 1980

F Rodeghiero, and T Barbui

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.

UI	MeSH Term	Description	Entries
D011485	Protein Binding	The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.	Plasma Protein Binding Capacity,Binding, Protein
D004591	Electrophoresis, Polyacrylamide Gel	Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.	Polyacrylamide Gel Electrophoresis,SDS-PAGE,Sodium Dodecyl Sulfate-PAGE,Gel Electrophoresis, Polyacrylamide,SDS PAGE,Sodium Dodecyl Sulfate PAGE,Sodium Dodecyl Sulfate-PAGEs
D004789	Enzyme Activation	Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme.	Activation, Enzyme,Activations, Enzyme,Enzyme Activations
D005176	Factor XIII	A fibrin-stabilizing plasma enzyme (TRANSGLUTAMINASES) that is activated by THROMBIN and CALCIUM to form FACTOR XIIIA. It is important for stabilizing the formation of the fibrin polymer (clot) which culminates the coagulation cascade.	Coagulation Factor XIII,Factor XIII Transamidase,Fibrin Stabilizing Factor,Fibrinase,Laki-Lorand Factor,Blood Coagulation Factor XIII,Factor 13,Factor Thirteen,Laki Lorand Factor,Factor XIII, Coagulation,Stabilizing Factor, Fibrin,Transamidase, Factor XIII,XIII, Coagulation Factor
D005177	Factor XIII Deficiency	A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis.	Deficiency, Factor 13,Deficiency, Factor Thirteen,Deficiency, Factor XIII,Factor 13 Deficiency,Factor Thirteen Deficiency,Deficiencies, Factor 13,Deficiencies, Factor Thirteen,Deficiencies, Factor XIII,Factor 13 Deficiencies,Factor Thirteen Deficiencies,Factor XIII Deficiencies
D005337	Fibrin	A protein derived from FIBRINOGEN in the presence of THROMBIN, which forms part of the blood clot.	Antithrombin I
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013917	Thrombin	An enzyme formed from PROTHROMBIN that converts FIBRINOGEN to FIBRIN.	Thrombase,Thrombin JMI,Thrombin-JMI,Thrombinar,Thrombostat,alpha-Thrombin,beta,gamma-Thrombin,beta-Thrombin,gamma-Thrombin,JMI, Thrombin