Structure and Function of Fibrinogen: Insights from Dysfibrinogens

Introduction: Hereditary dysfibrinogen is a fibrinogen molecule that is unable to exert its physiological functions due to a structural alteration(s) determined at the gene level. The functions may be inherent in the molecule, as represented by fibrin gel formation, but may also include interactions with Ca2+ and other plasma proteins, such as thrombin, t-PA and plasmin(ogen). This chapter focuses on the structure-function relationships of hereditary dysfibrinogens elucidated at the molecular level, their relevance to and insights into the constitutive functions of normal fibrinogen and fibrin, and their clinical implications. For these aims, the structure-function relationships will be discussed first in accordance with the successive steps of fibrin gel formation, then, with interactions with other substances, such as Ca2+, thrombin, and plasmin on the basis of the data provided from representative molecules.