2cui

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[[Image:2cui.png|left|200px]]
 
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{{STRUCTURE_2cui| PDB=2cui | SCENE= }}
{{STRUCTURE_2cui| PDB=2cui | SCENE= }}
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===Solution structure of the 31st fibronectin type III domain of the human tenascin X===
===Solution structure of the 31st fibronectin type III domain of the human tenascin X===
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==Disease==
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[[http://www.uniprot.org/uniprot/TENX_HUMAN TENX_HUMAN]] Defects in TNXB are the cause of tenascin-X deficiency (TNXD) [MIM:[http://omim.org/entry/606408 606408]]. TNXD leads to an Ehlers-Danlos-like syndrome characterized by hyperextensible skin, hypermobile joints, and tissue fragility. Tenascin-X-deficient patients, however, lack atrophic scars, a major diagnostic criteria for classic Ehlers-Danlos. Delayed wound healing, which is also common in classic EDS, is only present in a subset of patients.
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==Function==
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[[http://www.uniprot.org/uniprot/TENX_HUMAN TENX_HUMAN]] Appears to mediate interactions between cells and the extracellular matrix. Substrate-adhesion molecule that appears to inhibit cell migration. Accelerates collagen fibril formation. May play a role in supporting the growth of epithelial tumors.<ref>PMID:17033827</ref>
==About this Structure==
==About this Structure==
[[2cui]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2CUI OCA].
[[2cui]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2CUI OCA].
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==Reference==
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<references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Inoue, M.]]
[[Category: Inoue, M.]]

Revision as of 10:35, 24 March 2013

Template:STRUCTURE 2cui

Contents

Solution structure of the 31st fibronectin type III domain of the human tenascin X

Disease

[TENX_HUMAN] Defects in TNXB are the cause of tenascin-X deficiency (TNXD) [MIM:606408]. TNXD leads to an Ehlers-Danlos-like syndrome characterized by hyperextensible skin, hypermobile joints, and tissue fragility. Tenascin-X-deficient patients, however, lack atrophic scars, a major diagnostic criteria for classic Ehlers-Danlos. Delayed wound healing, which is also common in classic EDS, is only present in a subset of patients.

Function

[TENX_HUMAN] Appears to mediate interactions between cells and the extracellular matrix. Substrate-adhesion molecule that appears to inhibit cell migration. Accelerates collagen fibril formation. May play a role in supporting the growth of epithelial tumors.[1]

About this Structure

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

Reference

  1. Egging D, van den Berkmortel F, Taylor G, Bristow J, Schalkwijk J. Interactions of human tenascin-X domains with dermal extracellular matrix molecules. Arch Dermatol Res. 2007 Jan;298(8):389-96. Epub 2006 Oct 11. PMID:17033827 doi:10.1007/s00403-006-0706-9

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