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

Structural highlights

2cui is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:Solution NMR
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT, TOPSAN

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]

Evolutionary Conservation

 

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

References

  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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