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Blood Coagulation Factor XIII: A Multifunctional Transglutaminase

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Transglutaminases

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Abstract

Factor XIII is a pro-enzyme of plasma transglutaminase consisting of two enzymatic A subunits and two non-catalytic B subunits, and platelet transglutaminase consisting of two enzymatic A subunits. FXIII plays a critical role in the generation of a stable hemostatic plug, wound healing, maintaining pregnancy, angiogenesis, apoptosis and bacterial immobilization. FXIII catalyzes intermolecular cross-linking reactions between fibrin monomers and α2-antiplasmin. These reactions increase the mechanical strength of the fibrin clot and its resistance to proteolytic degradation. Congenital FXIII deficiency is a rare autosomal recessive disorder, most cases of which are caused by defects in the FXIII-A gene, leading to a bleeding tendency. An autoimmune hemophilia-like disease is caused by anti-FXIII antibodies. Platelet surface FXIII-A2 is involved in fibrin translocation to lipid rafts and outside-in signaling, leading to clot retraction. FXIII-A2-mediated protein cross-linking is associated with assembly of the extracellular matrix on a variety of cell surfaces in physiological events such as differentiation.

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Acknowledgments

We thank Dr A. Ichinose (Yamagata University School of Medicine) for helpful discussion.

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Authors and Affiliations

  1. Laboratory of Biomembrane, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan

    Moyuru Hayashi & Kohji Kasahara

Authors
  1. Moyuru Hayashi
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  2. Kohji Kasahara
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Correspondence to Kohji Kasahara .

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Editors and Affiliations

  1. Grad Sch of Pharmaceutical Sciences, Nagoya University, Nagoya, Aichi, Japan

    Kiyotaka Hitomi

  2. Micro-Signaling Regulation Technol. Unit, RIKEN Center for Life Sci. Technologies, Wako, Saitama, Japan

    Soichi Kojima

  3. Dept. Biochemistry and Molecular Biology, University of Debrecen, Debrecen, Hungary

    Laszlo Fesus

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Hayashi, M., Kasahara, K. (2015). Blood Coagulation Factor XIII: A Multifunctional Transglutaminase. In: Hitomi, K., Kojima, S., Fesus, L. (eds) Transglutaminases. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55825-5_15

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