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Gene Review

TNXA  -  tenascin XA (pseudogene)

Homo sapiens

Synonyms: D6S103E, HXBL, TN-XA, TNX, XA
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Disease relevance of TNXA


High impact information on TNXA

  • We describe a new contiguous-gene syndrome, involving the CYP21B and TNX genes, that results in 21-hydroxylase deficiency and a connective-tissue disorder consisting of skin and joint hyperextensibility, vascular fragility and poor wound healing [6].
  • The abundant expression of TNX in connective tissues is consistent with a role in EDS, and our patient's skin fibroblasts do not synthesize TNX protein in vitro or in vivo [6].
  • His paternal allele carries a novel deletion arising from recombination between TNX and its partial duplicate gene, XA, which precludes TNX synthesis [6].
  • Human populations are endowed with a sophisticated genetic diversity of complement C4 and its flanking genes RP, CYP21, and TNX in the RCCX modules of the major histocompatibility complex class III region [7].
  • To determine how this locus was duplicated, we sequenced the DNA at the duplication boundaries and the 7 kb between P450c21A and C4B comprising the XA locus [8].

Chemical compound and disease context of TNXA


Biological context of TNXA


Anatomical context of TNXA

  • Nevertheless, XA is transcribed into a stable 2.6-kb polyadenylated RNA that is expressed uniquely in the adrenal gland [8].
  • This complex locus is duplicated into A and B loci, so that the organization is 5'-C4A-21A-XA-C4B-21B-XB-3'. Although this duplication event truncated the 65-kb X(B) gene to a 4.5-kb XA gene, the XA gene is transcriptionally active in the adrenal cortex [11].
  • Although the precise role of TNX in the pathogenesis of EDS is uncertain, this patient's findings suggest a unique and essential role for TNX in connective-tissue structure and function [6].
  • Skin of TNX deficient patients shows abnormal elastic fibers and reduced collagen deposition [1].
  • Tenascin-X (TNX) is a large 450 kDa extracellular matrix protein expressed in a variety of tissues including skin, joints and blood vessels [1].

Associations of TNXA with chemical compounds


Other interactions of TNXA

  • The sequences beyond--1524 nt may be completely different as the C4 genes at RCCX module I have RPI-specific sequences, while those at Modules II, III and IV have TNXA-specific sequences [14].
  • PCR-based detection of the CYP21 deletion and TNXA/TNXB hybrid in the RCCX module [15].

Analytical, diagnostic and therapeutic context of TNXA

  • Absence of TNX mRNA and protein in the proband, mapping of the TNX gene and HLA typing of this family suggest recessive inheritance of TNX deficiency and connective-tissue disease [6].
  • RESULTS: A single intraperitoneal injection of 8 mg/kg TNX reduced the blood perfusion in FSaII tumors by 53% at 2 hours after injection [2].
  • METHODS AND MATERIALS: Human dermal microvascular endothelial cells were cultured in vitro and exposed to TNX under various combinations of aerobic, hypoxic, neutral, or acidic conditions, and levels of activated JNK MAP kinase were assessed by Western blotting [2].
  • The effect of adsorption filtration of leukocyte-reduced single donor platelet (SDP) units collected on a CS-3000+ with a TNX chamber was compared to filtration of SDP units prepared via an isoradial chamber [16].
  • Serum levels of TNX were analysed using high performance liquid chromatography [17].


  1. Interactions of human tenascin-X domains with dermal extracellular matrix molecules. Egging, D., van den Berkmortel, F., Taylor, G., Bristow, J., Schalkwijk, J. Arch. Dermatol. Res. (2007) [Pubmed]
  2. Preferential action of arsenic trioxide in solid-tumor microenvironment enhances radiation therapy. Griffin, R.J., Williams, B.W., Park, H.J., Song, C.W. Int. J. Radiat. Oncol. Biol. Phys. (2005) [Pubmed]
  3. Toxicity of the explosive metabolites hexahydro-1,3,5-trinitroso-1,3,5-triazine (TNX) and hexahydro-1-nitroso-3,5-dinitro-1,3,5-triazine (MNX) to the earthworm Eisenia fetida. Zhang, B., Kendall, R.J., Anderson, T.A. Chemosphere (2006) [Pubmed]
  4. Nervus terminalis lesions: II. Enhancement of lordosis induced by tactile stimulation in the hamster. Wirsig-Wiechmann, C.R. Physiol. Behav. (1997) [Pubmed]
  5. A clinical and cardiovascular survey of Ehlers-Danlos syndrome patients with complete deficiency of tenascin-X. Peeters, A.C., Kucharekova, M., Timmermans, J., van den Berkmortel, F.W., Boers, G.H., Nováková, I.R., Egging, D., den Heijer, M., Schalkwijk, J. The Netherlands journal of medicine. (2004) [Pubmed]
  6. Tenascin-X deficiency is associated with Ehlers-Danlos syndrome. Burch, G.H., Gong, Y., Liu, W., Dettman, R.W., Curry, C.J., Smith, L., Miller, W.L., Bristow, J. Nat. Genet. (1997) [Pubmed]
  7. Genetic sophistication of human complement components C4A and C4B and RP-C4-CYP21-TNX (RCCX) modules in the major histocompatibility complex. Chung, E.K., Yang, Y., Rennebohm, R.M., Lokki, M.L., Higgins, G.C., Jones, K.N., Zhou, B., Blanchong, C.A., Yu, C.Y. Am. J. Hum. Genet. (2002) [Pubmed]
  8. Mechanism and consequences of the duplication of the human C4/P450c21/gene X locus. Gitelman, S.E., Bristow, J., Miller, W.L. Mol. Cell. Biol. (1992) [Pubmed]
  9. Modular variations of the human major histocompatibility complex class III genes for serine/threonine kinase RP, complement component C4, steroid 21-hydroxylase CYP21, and tenascin TNX (the RCCX module). A mechanism for gene deletions and disease associations. Yang, Z., Mendoza, A.R., Welch, T.R., Zipf, W.B., Yu, C.Y. J. Biol. Chem. (1999) [Pubmed]
  10. Structure and genetics of the partially duplicated gene RP located immediately upstream of the complement C4A and the C4B genes in the HLA class III region. Molecular cloning, exon-intron structure, composite retroposon, and breakpoint of gene duplication. Shen, L., Wu, L.C., Sanlioglu, S., Chen, R., Mendoza, A.R., Dangel, A.W., Carroll, M.C., Zipf, W.B., Yu, C.Y. J. Biol. Chem. (1994) [Pubmed]
  11. Sequences promoting the transcription of the human XA gene overlapping P450c21A correctly predict the presence of a novel, adrenal-specific, truncated form of tenascin-X. Tee, M.K., Thomson, A.A., Bristow, J., Miller, W.L. Genomics (1995) [Pubmed]
  12. Organizations and gene duplications of the human and mouse MHC complement gene clusters. Yang, Z., Yu, C.Y. Exp. Clin. Immunogenet. (2000) [Pubmed]
  13. Evaluating the bioavailability of explosive metabolites, hexahydro-1-nitroso-3,5-dinitro-1,3,5-triazine (MNX) and hexahydro-1,3,5-trinitroso-1,3,5-triazine (TNX), in soils using passive sampling devices. Zhang, B., Smith, P.N., Anderson, T.A. Journal of chromatography. A. (2006) [Pubmed]
  14. Genetic, structural and functional diversities of human complement components C4A and C4B and their mouse homologues, Slp and C4. Blanchong, C.A., Chung, E.K., Rupert, K.L., Yang, Y., Yang, Z., Zhou, B., Moulds, J.M., Yu, C.Y. Int. Immunopharmacol. (2001) [Pubmed]
  15. PCR-based detection of the CYP21 deletion and TNXA/TNXB hybrid in the RCCX module. Lee, H.H., Lee, Y.J., Lin, C.Y. Genomics (2004) [Pubmed]
  16. Adsorption filtration of leukocyte-reduced single donor platelet units. AuBuchon, J.P., Burbank, A., Webber, S., O'Neill, M. Vox Sang. (1994) [Pubmed]
  17. Bioequivalence of two oral dosage forms prepared from different polymorphic modifications of tenoxicam. Salem, M.S., Alkaysi, H.N., Gharaibeh, A.M., Gharaibeh, N.M., Badwan, A.A. Journal of clinical pharmacy and therapeutics. (1994) [Pubmed]
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