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Biological Chemistry

Editor-in-Chief: Brüne, Bernhard

Editorial Board: Buchner, Johannes / Lei, Ming / Ludwig, Stephan / Thomas, Douglas D. / Turk, Boris / Wittinghofer, Alfred

IMPACT FACTOR 2018: 3.014
5-year IMPACT FACTOR: 3.162

CiteScore 2018: 3.09

SCImago Journal Rank (SJR) 2018: 1.482
Source Normalized Impact per Paper (SNIP) 2018: 0.820

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Activation mechanisms of coagulation factor IX

Thomas Zögg
  • Department of Molecular Biology, Division of Structural Biology, University of Salzburg, Billrothstraße 11, A-5020 Salzburg, Austria
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Hans Brandstetter
  • Department of Molecular Biology, Division of Structural Biology, University of Salzburg, Billrothstraße 11, A-5020 Salzburg, Austria
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2009-04-10 | DOI: https://doi.org/10.1515/BC.2009.057


Blood haemostasis is accomplished by a complex network of coagulatory and fibrinolytic processes. These processes have to be delicately balanced, as clinically manifested by bleeding disorders, such as haemophilia A and B. These disorders are caused by defects in coagulation factor VIII and factor IX, respectively. Following a dual strategy, we emphasise on the one hand principles conserved in most coagulation enzymes, thus mirroring much of the underlying complexity in haemostasis; on the other hand, we identify enzymatic properties of the factor IXa-factor VIIIa system (Xase) that distinguish this proteolytic machine from other components of the coagulation system. While the exact mechanisms of its activity modulation remain baffling until today, superactive factor IX mutants significantly improve our current understanding and serve as a specific and testable model of Xase action.

Keywords: Christmas factor; disorder-order transition; gatekeeper model; haemostasis; reversible activity modulation; zymogen

About the article

Corresponding author

Received: 2009-02-02

Accepted: 2009-03-13

Published Online: 2009-04-10

Published in Print: 2009-01-01

Citation Information: Biological Chemistry, Volume 390, Issue 5/6, Pages 391–400, ISSN (Online) 1437-4315, ISSN (Print) 1431-6730, DOI: https://doi.org/10.1515/BC.2009.057.

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