Fibrinogen Mitaka 11 : A Hereditary Dysfibrinogen With Defective Thrombin


A new type of Aa Glu-I 1 to Gly substitution has been identified in a congenitally abnormal fibrinogen, fibrinogen Mitaka ll, derived from a 14-year-old female suffering from easy bruising since childhood. Plasma of the patient and fibrinogen purified therefrom were found to clot slowly by thrombin but in a normal fashion by ancrod, a thrombin-like snake venom enzyme. The ancrod-clotted fibrin gels were normally solid and turbid, whereas the thrombin-clotted gels were initially fragile and transparent but became gradually normalized during further incubation. On reversephase high-performance liquid chromatography, there was an additional peptide group eluted distinctly later than the corresponding normal fibrinopeptide A in the clot-liquor of the patient's samples. Sequence analysis of these aberrant peptides and isolated Aa chains of the patient's

Cite this paper

@inproceedings{Niwa2003FibrinogenM1, title={Fibrinogen Mitaka 11 : A Hereditary Dysfibrinogen With Defective Thrombin}, author={Kazuki Niwa and Akihiro Yaginuma and Makoto Nakanishi and Yasuo Wada and Teruko Sugo and Shinji Asakura and Nobuo Watanabe and Michio Matsuda}, year={2003} }