1evu
From Proteopedia
(Difference between revisions)
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| - | [[Image:1evu.png|left|200px]] | ||
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{{STRUCTURE_1evu| PDB=1evu | SCENE= }} | {{STRUCTURE_1evu| PDB=1evu | SCENE= }} | ||
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===HUMAN FACTOR XIII WITH CALCIUM BOUND IN THE ION SITE=== | ===HUMAN FACTOR XIII WITH CALCIUM BOUND IN THE ION SITE=== | ||
| + | ==Disease== | ||
| + | [[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== | ||
| + | [[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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==See Also== | ==See Also== | ||
*[[Factor XIII|Factor XIII]] | *[[Factor XIII|Factor XIII]] | ||
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| + | ==Reference== | ||
| + | <references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Protein-glutamine gamma-glutamyltransferase]] | [[Category: Protein-glutamine gamma-glutamyltransferase]] | ||
Revision as of 18:51, 24 March 2013
Contents |
HUMAN FACTOR XIII WITH CALCIUM BOUND IN THE ION SITE
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
1evu is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
