[Fibrinogen Zürich I: defective fibrinopeptide-A release]. 1977

V Hofmann, and W P Gati, and P W Straub

Fibrinogen Zurich I (FZI) is characterized by delayed fibrin monomer aggregation. It has been previously shown that the patient fibrinogen is composed of two populations of molecules, one aggregating normally and the other not aggregating at all but interfering with the aggregation of the normal population. By use of a radioimmunoassay for fibrinopeptide A (FPA), only approximately half of the expected FPA could be recovered after thrombin and Defibrase proteolysis. Even after exhaustive proteolysis with 2 thrombin units of Defibrase, no further release of FPA could be obtained. The defective FPA release was confirmed by measurement of the N-terminal Gly/Tyr ratio. It may be assumed that the abnormal aggregation of one fraction of FZI is due to the defective FPA release from this fraction.

UI MeSH Term Description Entries
D012103 Batroxobin A proteolytic enzyme obtained from the venom of fer-de-lance (BOTHROPS ATROX). It is used as a plasma clotting agent for fibrinogen and for the detection of fibrinogen degradation products. The presence of heparin does not interfere with the clotting test. Hemocoagulase is a mixture containing batroxobin and factor X activator. EC 3.4.21.-. Defibrol,Hemocoagulase,Defibrase,Reptilase,Reptilase-DEF,Reptilase DEF
D005340 Fibrinogen Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products. Coagulation Factor I,Factor I,Blood Coagulation Factor I,gamma-Fibrinogen,Factor I, Coagulation,gamma Fibrinogen
D005342 Fibrinolysis The natural enzymatic dissolution of FIBRIN. Fibrinolyses
D005344 Fibrinopeptide A Two small peptide chains removed from the N-terminal segment of the alpha chains of fibrinogen by the action of thrombin during the blood coagulation process. Each peptide chain contains 18 amino acid residues. In vivo, fibrinopeptide A is used as a marker to determine the rate of conversion of fibrinogen to fibrin by thrombin. Fibrinopeptides A
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

Related Publications

V Hofmann, and W P Gati, and P W Straub
February 1985, Thrombosis research,
V Hofmann, and W P Gati, and P W Straub
May 1974, The Journal of clinical investigation,
V Hofmann, and W P Gati, and P W Straub
January 1980, Thrombosis research,
V Hofmann, and W P Gati, and P W Straub
January 1985, Thrombosis research,
V Hofmann, and W P Gati, and P W Straub
June 1983, Annals of the New York Academy of Sciences,
V Hofmann, and W P Gati, and P W Straub
May 1980, Scandinavian journal of clinical and laboratory investigation,
V Hofmann, and W P Gati, and P W Straub
September 1980, British journal of haematology,
V Hofmann, and W P Gati, and P W Straub
September 1971, The Journal of clinical investigation,
Copied contents to your clipboard!