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Structural Alterations in Hereditary Dysfibrinogens

[ Vol. 3 , Issue. 3 ]

Author(s):

Teruko Sugo, Yoichi Sakata and Michio Matsuda   Pages 239 - 247 ( 9 )

Abstract:


Dysfibrinogens can be grossly divided in two groups: (1) defective thrombin-catalyzed conversion of fibrinogen molecules to fibrin monomers, and (2) defective fibrin polymerization due to structural alterations in polymerization sites, that include “A” and “a” sites, end-to-end D:D abutment surfaces, and lateral association sites involving the carboxyl terminal region of the fibrin α-chain. Recently, a number of mutations in the fibrinogen genes have been identified, and many of these encode changes that occur in regions of fibrinogen that have been elucidated by high-resolution structural studies. Here we focus on the structure-function relationships of fibrinogen that can be inferred from studies involving these abnormal molecules.

Keywords:

dysfibrinogens, gamma275, alpha-16, dysfibrogenernias, d:d interface

Affiliation:

Division of Cell and Molecular Medicine, Center for Molecular Medicine, Jichi Medical School, Yakushiji 3311-1, Tochigi, 329-0498, Japan



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