1ggy

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Template:STRUCTURE 1ggy

Contents 1 HUMAN FACTOR XIII WITH YTTERBIUM BOUND IN THE ION SITE 2 Disease 3 Function 4 About this Structure 5 See Also 6 Reference

HUMAN FACTOR XIII WITH YTTERBIUM BOUND IN THE ION SITE

Template:ABSTRACT PUBMED 9988734

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

1ggy is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

• Factor XIII

Reference

• Fox BA, Yee VC, Pedersen LC, Le Trong I, Bishop PD, Stenkamp RE, Teller DC. Identification of the calcium binding site and a novel ytterbium site in blood coagulation factor XIII by x-ray crystallography. J Biol Chem. 1999 Feb 19;274(8):4917-23. PMID:9988734

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