Fibrinogen Kawaguchi: an abnormal fibrinogen characterized by defective release of fibrinopeptide A.
A congenital dysfibrinogenemia was found in a 32-year-old asymptomatic female and her immediate family. The propositus, apparently a heterozygote for the abnormality, characteristically showed defective release of fibrinopeptide A from half of her fibrinogen molecules. No fibrinopeptide A was cleaved off from the isolated abnormal molecule by thrombin or snake venoms (Reptilase and Ancrod) as evidenced by radioimmunoassay, high performance liquid chromatography and determination of the NH2-terminal amino acids. The abnormal fibrinogen formed a solid gel solely by the release of fibrinopeptide B upon incubation with thrombin. We provisionally designate this abnormal fibrinogen as "Fibrinogen Kawaguchi", although possible identity with other abnormal fibrinogens is not excluded.
D000328:Adult; D000595:Amino Acid Sequence; D001778:Blood Coagulation Disorders / Q000097:blood / Q000235:genetics*; D005260:Female; D005337:Fibrin / Q000378:metabolism; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005340:Fibrinogen / Q000378:metabolism*; D015241:Fibrinogens, Abnormal; D005344:Fibrinopeptide A / Q000378:metabolism; D006487:Hemostasis; D006801:Humans; D010375:Pedigree; D013918:Thrombin Time
D005338:Fibrin Fibrinogen Degradation Products; D015241:Fibrinogens, Abnormal; C045162:fibrinogen Kawaguchi; D005344:Fibrinopeptide A; D005337:Fibrin; D005340:Fibrinogen