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Expression of full-length utrophin prevents muscular dystrophy in mdx mice

Bibliographic reference Tinsley, J ; Kahn, David ; Deconinck, N. ; Fisher, R ; Phelps, S ; et. al. Expression of full-length utrophin prevents muscular dystrophy in mdx mice. In: Nature Medicine, Vol. 4, no. 12, p. 1441-1444 (1998)
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  1. Emery, A.E.H. in Duchenne Muscular Dystrophy (Oxford Medical Publications, Oxford, 1993).
  2. Tinsley J. M., Blake D. J., Roche A., Fairbrother U., Riss J., Byth B. C., Knight A. E., Kendrick-Jones J., Suthers G. K., Love D. R., Edwards Y. H., Davies K. E., Primary structure of dystrophin-related protein, 10.1038/360591a0
  3. Love D.R., Proc. Natl. Acad. Sci. USA, 88, 3243 (1991)
  4. Khurana Tejvir S., Watkins Simon C., Chafey Philippe, Chelly Jamel, Tomé Fernando M.S., Fardeau Michel, Kaplan Jean-Claude, Kunkel Louis M., Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle, 10.1016/0960-8966(91)90023-l
  5. Deconinck N., Nature Med., 3, 1216 (1997)
  6. Tinsley Jonathon M., Potter Allyson C., Phelps Steven R., Fisher Rosie, Trickett Jeffrey I., Davies Kay E., Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene, 10.1038/384349a0
  7. Brennan K.J., J. Biol. Chem., 268, 719 (1993)
  8. Bulfield G., Proc. Natl. Acad. Sci. USA, 81, 1189 (1984)
  9. Blake D.J., Proc. Natl. Acad. Sc.i USA, 92, 3697 (1995)
  10. Straub V., Curr. Opin. Neurol., 10, 168 (1997)
  11. Sadoulet-Puccio H. M., Rajala M., Kunkel L. M., Dystrobrevin and dystrophin: An interaction through coiled-coil motifs, 10.1073/pnas.94.23.12413
  12. Suzuki A., Mammalian alpha 1- and beta 1-syntrophin bind to the alternative splice- prone region of the dystrophin COOH terminus, 10.1083/jcb.128.3.373
  13. Blake D. J., Nawrotzki R., Loh N. Y., Gorecki D. C., Davies K. E.,  -dystrobrevin, a member of the dystrophin-related protein family, 10.1073/pnas.95.1.241
  14. Moens P., J. Muscle Res. Cell. Motil., 14, 446 (1993)
  15. Petrof B.J., Proc. Natl. Acad. Sci. USA, 90, 3710 (1993)
  16. Weller Boaz, Karpati George, Carpenter Stirling, Dystrophin-deficient mdx muscle fibers are preferentially vulnerable to necrosis induced by experimental lengthening contractions, 10.1016/0022-510x(90)90005-8
  17. Stedman H. H., Sweeney H. L., Shrager J. B., Maguire H. C., Panettieri R. A., Petrof B., Narusawa M., Leferovich J. M., Sladky J. T., Kelly A. M., The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy, 10.1038/352536a0
  18. Nguyen T. M., Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines, 10.1083/jcb.115.6.1695
  19. Carlson C.G., Muscle Nerve, 13, 480 (1990)
  20. Deconinck Anne E., Potter Allyson C., Tinsley Jonathon M., Wood Sarah J., Vater Ruth, Young Carol, Metzinger Laurent, Vincent Angela, Slater Clarke R., Davies Kay E., Postsynaptic Abnormalities at the Neuromuscular Junctions of Utrophin-deficient Mice, 10.1083/jcb.136.4.883