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Tenascin-X (TNXB)
- Target
- TNXB
- Molecular classification
- Extracellular matrix glycoprotein, Matricellular protein, Other (not a receptor, enzyme, ion channel, transporter, or transcription factor)
01
Overview
Tenascin-X is a large extracellular matrix glycoprotein encoded by the TNXB gene, predominantly expressed in connective tissues such as skin, muscle, kidney, blood vessels, and digestive tract[1][2][3][4]. It plays a fundamental role in maintaining tissue structure, supporting the organization and maturation of collagen and elastic fibers, and modulating cell adhesion. Tenascin-X deficiency, due to gene mutations or haploinsufficiency, causes forms of Ehlers-Danlos syndrome marked by hypermobile joints, skin hyperextensibility, and other connective tissue deformities. Tenascin-X also regulates the bioavailability of transforming growth factor beta (TGF-β), affecting cell plasticity and possibly responses such as epithelial-to-mesenchymal transition. Unlike other tenascins, Tenascin-X is not known to serve as a receptor, enzyme, or drug target; research is ongoing into its molecular mechanisms in tissue repair and disease pathogenesis[1][2][3][4][5].
- Other names
- Tenascin-XTNXBHXBLTENXTN-XTNXB1TNXB2TNXBSXBXBSFlexillinHexabrachion-like proteinEDS3Growth-inhibiting protein 45Vesicoureteral reflux 8 (VUR8)
02
Mechanism of action
Not applicable
03
Biological functions
Extracellular matrix architecture (organizing and maintaining the structure of connective tissues)Regulation of collagen and elastic fibers (assembly and stability)Counter-adhesive properties (modulates cell adhesion)Regulation of TGF-β bioavailability and signaling (affects epithelial plasticity and matrix maturation)Matrix maturation during wound healing
04
Disease associations
Ehlers-Danlos syndrome (classic-like and hypermobile types) (mutations or deficiency linked to structural tissue disorders)Benign joint hypermobility syndrome (associated with reduced tenascin-X protein)Vesicoureteral reflux 8Congenital adrenal hyperplasia with TNXB deficiency (CAH-X)
05
Safety considerations
Not applicable
06
Interacting drugs
None identified
07
Biomarkers
Reduced tenascin-X protein (for diagnosing classical-like Ehlers-Danlos syndrome and possibly benign joint hypermobility syndrome)
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