4kty

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Template:STRUCTURE 4kty

Contents

Fibrin-stabilizing factor with a bound ligand

Template:ABSTRACT PUBMED 24115223

Disease

[F13A_HUMAN] Defects in F13A1 are the cause of factor XIII subunit A deficiency (FA13AD) [MIM:613225]. FA13AD is an autosomal recessive disorder characterized by a life-long bleeding tendency, impaired wound healing and spontaneous abortion in affected women.[1]

Function

[F13A_HUMAN] Factor XIII is activated by thrombin and calcium ion to a transglutaminase that catalyzes the formation of gamma-glutamyl-epsilon-lysine cross-links between fibrin chains, thus stabilizing the fibrin clot. Also cross-link alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin.

About this Structure

4kty is a 4 chain structure with sequence from Human. Full crystallographic information is available from OCA.

See Also

Reference

  • Stieler M, Weber J, Hils M, Kolb P, Heine A, Buchold C, Pasternack R, Klebe G. Structure of Active Coagulation Factor XIII Triggered by Calcium Binding: Basis for the Design of Next-Generation Anticoagulants. Angew Chem Int Ed Engl. 2013 Sep 20. doi: 10.1002/anie.201305133. PMID:24115223 doi:http://dx.doi.org/10.1002/anie.201305133
  1. ↑ Board P, Coggan M, Miloszewski K. Identification of a point mutation in factor XIII A subunit deficiency. Blood. 1992 Aug 15;80(4):937-41. PMID:1353995

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