1qrk

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[[Image:1qrk.png|left|200px]]
 
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{{STRUCTURE_1qrk| PDB=1qrk | SCENE= }}
{{STRUCTURE_1qrk| PDB=1qrk | SCENE= }}
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===HUMAN FACTOR XIII WITH STRONTIUM BOUND IN THE ION SITE===
===HUMAN FACTOR XIII WITH STRONTIUM BOUND IN THE ION SITE===
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{{ABSTRACT_PUBMED_9988734}}
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{{ABSTRACT_PUBMED_9988734}}
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==Disease==
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[[http://www.uniprot.org/uniprot/F13A_HUMAN F13A_HUMAN]] Defects in F13A1 are the cause of factor XIII subunit A deficiency (FA13AD) [MIM:[http://omim.org/entry/613225 613225]]. FA13AD is an autosomal recessive disorder characterized by a life-long bleeding tendency, impaired wound healing and spontaneous abortion in affected women.<ref>PMID:1353995</ref>
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==Function==
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[[http://www.uniprot.org/uniprot/F13A_HUMAN 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==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:009988734</ref><references group="xtra"/>
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<ref group="xtra">PMID:009988734</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Protein-glutamine gamma-glutamyltransferase]]
[[Category: Protein-glutamine gamma-glutamyltransferase]]

Revision as of 02:42, 25 March 2013

Template:STRUCTURE 1qrk

Contents

HUMAN FACTOR XIII WITH STRONTIUM 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

1qrk is a 2 chain structure with sequence from Homo sapiens. This structure supersedes the now removed PDB entry 1bl2. Full crystallographic information is available from OCA.

See Also

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

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