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Gene Review

Utrn  -  utrophin

Mus musculus

Synonyms: AA589569, DRP, Dmdl, G-utrophin
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Disease relevance of Utrn


High impact information on Utrn


Chemical compound and disease context of Utrn


Biological context of Utrn


Anatomical context of Utrn


Associations of Utrn with chemical compounds

  • In the present study, we have therefore focused on the effect of agrin on utrophin expression in cultured C2 myotubes [18].
  • In response to Torpedo-, muscle-, or nerve-derived agrin, we observed a significant 2-fold increase in utrophin mRNAs [18].
  • The transduced fibers were largely impermeable to the tracer dye Evans blue, suggesting that utrophin protects the surface membrane from breakage [19].
  • We injected an adenoviral vector expressing a synthetic utrophin into tibialis anterior muscles of newborn dogs affected with CXMD and examined transgene expression by RNA and protein analysis at 10, 30 and 60 days postinjection in cyclosporin-treated and -untreated animals [5].
  • We previously found that L-arginine, the substrate for nitric oxide synthase, significantly increased utrophin level in muscle and targeted it to the sarcolemma [20].

Physical interactions of Utrn


Co-localisations of Utrn


Regulatory relationships of Utrn

  • G-utrophin is expressed in brain sites generally different from the regions expressing beta-dystroglycan [12].
  • The utrophin promoter A is transcriptionally regulated in part by heregulin-mediated, extracellular signal-related kinase-dependent activation of the GABP(alpha/beta) transcription factor complex [2].
  • In addition, we showed that activation of cellular calpains by Ca2+ ionophore treatment reduces utrophin protein levels in muscle cells and that calpain inhibition prevents this Ca2+ -induced reduction in utrophin levels [24].
  • Interleukin 6 induces overexpression of the sarcolemmal utrophin in neonatal mdx skeletal muscle [25].
  • Furthermore, this increase in transcriptional activity in response to agrin resulted from a greater number of myonuclei expressing the 1.3-kilobase pair utrophin promoter-nlsLacZ construct [18].

Other interactions of Utrn


Analytical, diagnostic and therapeutic context of Utrn


  1. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Grady, R.M., Teng, H., Nichol, M.C., Cunningham, J.C., Wilkinson, R.S., Sanes, J.R. Cell (1997) [Pubmed]
  2. Heregulin ameliorates the dystrophic phenotype in mdx mice. Krag, T.O., Bogdanovich, S., Jensen, C.J., Fischer, M.D., Hansen-Schwartz, J., Javazon, E.H., Flake, A.W., Edvinsson, L., Khurana, T.S. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  3. Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene. Wakefield, P.M., Tinsley, J.M., Wood, M.J., Gilbert, R., Karpati, G., Davies, K.E. Gene Ther. (2000) [Pubmed]
  4. Expression of full-length utrophin prevents muscular dystrophy in mdx mice. Tinsley, J., Deconinck, N., Fisher, R., Kahn, D., Phelps, S., Gillis, J.M., Davies, K. Nat. Med. (1998) [Pubmed]
  5. Dystrophic phenotype of canine X-linked muscular dystrophy is mitigated by adenovirus-mediated utrophin gene transfer. Cerletti, M., Negri, T., Cozzi, F., Colpo, R., Andreetta, F., Croci, D., Davies, K.E., Cornelio, F., Pozza, O., Karpati, G., Gilbert, R., Mora, M. Gene Ther. (2003) [Pubmed]
  6. Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice. Rafael, J.A., Tinsley, J.M., Potter, A.C., Deconinck, A.E., Davies, K.E. Nat. Genet. (1998) [Pubmed]
  7. Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene. Tinsley, J.M., Potter, A.C., Phelps, S.R., Fisher, R., Trickett, J.I., Davies, K.E. Nature (1996) [Pubmed]
  8. Nitric oxide and l-arginine cause an accumulation of utrophin at the sarcolemma: a possible compensation for dystrophin loss in Duchenne muscular dystrophy. Chaubourt, E., Fossier, P., Baux, G., Leprince, C., Israël, M., De La Porte, S. Neurobiol. Dis. (1999) [Pubmed]
  9. Increased vulnerability to kainate-induced seizures in utrophin-knockout mice. Knuesel, I., Riban, V., Zuellig, R.A., Schaub, M.C., Grady, R.M., Sanes, J.R., Fritschy, J.M. Eur. J. Neurosci. (2002) [Pubmed]
  10. Full-length and short forms of utrophin, the dystrophin-related protein. Nguyen, T.M., Helliwell, T.R., Simmons, C., Winder, S.J., Kendrick-Jones, J., Davies, K.E., Morris, G.E. FEBS Lett. (1995) [Pubmed]
  11. Congenital myasthenic syndromes in two kinships with end-plate acetylcholine receptor and utrophin deficiency. Sieb, J.P., Dörfler, P., Tzartos, S., Wewer, U.M., Rüegg, M.A., Meyer, D., Baumann, I., Lindemuth, R., Jakschik, J., Ries, F. Neurology (1998) [Pubmed]
  12. G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain. Blake, D.J., Schofield, J.N., Zuellig, R.A., Górecki, D.C., Phelps, S.R., Barnard, E.A., Edwards, Y.H., Davies, K.E. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  13. Utrophin and dystrophin-associated glycoproteins in normal and dystrophin deficient cardiac muscle. Rivier, F., Robert, A., Royuela, M., Hugon, G., Bonet-Kerrache, A., Mornet, D. J. Muscle Res. Cell. Motil. (1999) [Pubmed]
  14. Genetic disruption of calcineurin improves skeletal muscle pathology and cardiac disease in a mouse model of limb-girdle muscular dystrophy. Parsons, S.A., Millay, D.P., Sargent, M.A., Naya, F.J., McNally, E.M., Sweeney, H.L., Molkentin, J.D. J. Biol. Chem. (2007) [Pubmed]
  15. A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle. Weir, A.P., Burton, E.A., Harrod, G., Davies, K.E. J. Biol. Chem. (2002) [Pubmed]
  16. Effect of beta-dystroglycan processing on utrophin/Dp116 anchorage in normal and mdx mouse Schwann cell membrane. Hnia, K., Hugon, G., Masmoudi, A., Mercier, J., Rivier, F., Mornet, D. Neuroscience (2006) [Pubmed]
  17. Efficient utrophin expression following adenovirus gene transfer in dystrophic muscle. Gilbert, R., Nalbanoglu, J., Tinsley, J.M., Massie, B., Davies, K.E., Karpati, G. Biochem. Biophys. Res. Commun. (1998) [Pubmed]
  18. Muscle and neural isoforms of agrin increase utrophin expression in cultured myotubes via a transcriptional regulatory mechanism. Gramolini, A.O., Burton, E.A., Tinsley, J.M., Ferns, M.J., Cartaud, A., Cartaud, J., Davies, K.E., Lunde, J.A., Jasmin, B.J. J. Biol. Chem. (1998) [Pubmed]
  19. Adenovirus-mediated utrophin gene transfer mitigates the dystrophic phenotype of mdx mouse muscles. Gilbert, R., Nalbantoglu, J., Petrof, B.J., Ebihara, S., Guibinga, G.H., Tinsley, J.M., Kamen, A., Massie, B., Davies, K.E., Karpati, G. Hum. Gene Ther. (1999) [Pubmed]
  20. L-arginine improves dystrophic phenotype in mdx mice. Voisin, V., Sébrié, C., Matecki, S., Yu, H., Gillet, B., Ramonatxo, M., Israël, M., De la Porte, S. Neurobiol. Dis. (2005) [Pubmed]
  21. Expression of utrophin A mRNA correlates with the oxidative capacity of skeletal muscle fiber types and is regulated by calcineurin/NFAT signaling. Chakkalakal, J.V., Stocksley, M.A., Harrison, M.A., Angus, L.M., Deschenes-Furry, J., St-Pierre, S., Megeney, L.A., Chin, E.R., Michel, R.N., Jasmin, B.J. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  22. Differential expression of syntrophins and analysis of alternatively spliced dystrophin transcripts in the mouse brain. Górecki, D.C., Abdulrazzak, H., Lukasiuk, K., Barnard, E.A. Eur. J. Neurosci. (1997) [Pubmed]
  23. Utrophin localization in normal and dystrophin-deficient heart. Pons, F., Robert, A., Fabbrizio, E., Hugon, G., Califano, J.C., Fehrentz, J.A., Martinez, J., Mornet, D. Circulation (1994) [Pubmed]
  24. Utrophin is a calpain substrate in muscle cells. Courdier-Fruh, I., Briguet, A. Muscle Nerve (2006) [Pubmed]
  25. Interleukin 6 induces overexpression of the sarcolemmal utrophin in neonatal mdx skeletal muscle. Fujimori, K., Itoh, Y., Yamamoto, K., Miyagoe-Suzuki, Y., Yuasa, K., Yoshizaki, K., Yamamoto, H., Takeda, S. Hum. Gene Ther. (2002) [Pubmed]
  26. Claudin-5 localizes to the lateral membranes of cardiomyocytes and is altered in utrophin/dystrophin-deficient cardiomyopathic mice. Sanford, J.L., Edwards, J.D., Mays, T.A., Gong, B., Merriam, A.P., Rafael-Fortney, J.A. J. Mol. Cell. Cardiol. (2005) [Pubmed]
  27. Induction of utrophin gene expression by heregulin in skeletal muscle cells: role of the N-box motif and GA binding protein. Gramolini, A.O., Angus, L.M., Schaeffer, L., Burton, E.A., Tinsley, J.M., Davies, K.E., Changeux, J.P., Jasmin, B.J. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  28. Characterization of dystrophin and utrophin diversity in the mouse. Lumeng, C.N., Phelps, S.F., Rafael, J.A., Cox, G.A., Hutchinson, T.L., Begy, C.R., Adkins, E., Wiltshire, R., Chamberlain, J.S. Hum. Mol. Genet. (1999) [Pubmed]
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