Primary Identifier | IPR033081 | Type | Family |
Short Name | TNX |
description | Tenascin-X (TNX) is a tenascin family member that contributes to matrix stability and is possibly involved in collagen fibril formation [, ]. Mutations in the TNX gene cause Ehlers-Danlos syndrome due to tenascin X deficiency (EDSTNXD), an Ehlers-Danlos-like syndrome characterised by hyperextensible skin, hypermobile joints, and tissue fragility [].Tenascins are extracellular matrix glycoproteins that act both as integrin ligands and as modifiers of fibronectin-integrin interactions to regulate cell adhesion, migration, proliferation and differentiation. Tenascins are usually composed of repeated epidermal growth factor (EGF)-like domains, fibronectin-type III (FNIII) domains and a C-terminal fibrinogen related domain (FReD) []. |