Fibrinogen Milano II: a congenital dysfibrinogenaemia associated with juvenile arterial and venous thrombosis.
A congenitally abnormal fibrinogen was isolated from blood of a young man with deep-vein thrombosis. Two other affected members of his family had three episodes of severe arterial thrombosis. The fibrinogen showed a delayed clotting by thrombin, but a normal clotting by Arvin, Reptilase, and prothrombin-staphylocoagulase complex. Analysis of the fibrinopeptides A and B by High Performance Liquid Chromatography did not reveal an abnormal peptide structure. The rate of release of A and B peptides by thrombin was strongly delayed, whereas the rate of release of fibrinopeptide A by Arvin appeared to be normal. The fibrin polymerization rate was normal. Interactions between the abnormal fibrinogen, platelets and the fibrinolytic system were also normal. Evidence is presented that the defective interaction between fibrinogen Milano II and thrombin is associated with a defective binding of thrombin to the fibrin moiety of the abnormal fibrinogen.
Haverkate
F
F
Koopman
J
J
Kluft
C
C
D'Angelo
A
A
Cattaneo
M
M
Mannucci
P M
PM
D000328:Adult; D001778:Blood Coagulation Disorders / Q000150:complications / Q000151:congenital / Q000235:genetics*; D005337:Fibrin / Q000378:metabolism; D005340:Fibrinogen / Q000302:isolation & purification*; D015241:Fibrinogens, Abnormal; D005344:Fibrinopeptide A / Q000302:isolation & purification; D005345:Fibrinopeptide B / Q000302:isolation & purification; D006801:Humans; D008297:Male; D010375:Pedigree; D010974:Platelet Aggregation; D013917:Thrombin / Q000378:metabolism; D013924:Thrombophlebitis / Q000150:complications / Q000235:genetics; D013927:Thrombosis / Q000150:complications* / Q000235:genetics
D015241:Fibrinogens, Abnormal; C048634:fibrinogen Milano II; D005344:Fibrinopeptide A; D005345:Fibrinopeptide B; D005337:Fibrin; D005340:Fibrinogen; D013917:Thrombin