The (Patho)physiology of Fibrinogen gamma'

Fraser L. Macrae, Marco M. Domingues, Alessandro Casini, Robert A. S. Ariens*

*Corresponding author for this work

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Abstract

Fibrinogen ?' is a splice variant of the fibrinogen ?-chain, which leads to a negatively charged extension at the C-terminus of the ?-chain. In fibrinogen, the splice variant appears mainly as a heterodimer with the common ?A chain, as ?A/?' fibrinogen. This variant has been shown to modulate thrombin and factor XIII (FXIII) activity, influence clot architecture, and lack a platelet-binding site. Clinically ?A/?' fibrinogen levels have been associated with arterial and venous thromboses, indicating that the functional effects of ?A/?' fibrinogen may contribute to the pathology of thrombosis. In view of the fact that the splice variant has several functional effects and is found so far in all individuals, this review provides an up-to-date summary of the key biologic aspects of this fibrinogen variant and discusses any inconsistencies in current reports. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
Original languageEnglish
Pages (from-to)344-355
JournalSeminars in Thrombosis and Hemostasis
Volume42
Issue number4
DOIs
Publication statusPublished - Jun 2016

Keywords

  • fibrinogen
  • venous
  • arterial
  • thrombosis
  • FXIII
  • thrombin
  • clot structure

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