tenascin-X; TN-X; hexabrachion-like protein (TNXB HXBL TNX TNXB1 TNXB2 XB)
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Function
- appears to mediate interactions between cells & the extracellular matrix
- substrate-adhesion molecule that appears to inhibit cell migration
- accelerates collagen fibril formation
- interacts with collagens
- interacts with tropoelastin
Structure
- homotrimer
- belongs to the tenascin family
- contains 19 EGF-like domains
- contains 1 fibrinogen C-terminal domain
- contains 32 fibronectin F3 modules
- interacts with collagens & tropoelastin via its 29th fibronectin F3 module
Compartment
Alternative splicing
named isoforms=3; XB; XB-short
Expression
- highly expressed in fetal adrenal, in fetal testis, fetal smooth muslce, striated muscle & cardiac muscle
- isoform XB-short is only expressed in the adrenal gland
- expression levels are lower in adults than in children
Pathology
- may play a role in supporting growth of epithelial tumors
- association with congenital adrenal hyperplasia
- defects in TNXB are the cause of tenascin-X deficiency
Genetics
- there are two genes for tenascin-X: TNXA & TNXB
- TNXA is a partial gene which can sometimes recombine with TNXB