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

X chromosome-linked muscular dystrophy (mdx) in the mouse

Bulfield, G., Siller, W. G., Wight, P. A., & Moore, K. J. (1984) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.81.4.1189 · Cited by 1,765

Dystrophin protects the sarcolemma from stresses developed during muscle contraction

Petrof, B. J., Shrager, J. B., Stedman, H. H., Kelly, A. M., & Sweeney, H. L. (1993) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.90.8.3710 · Cited by 1,526

Duchenne Muscular Dystrophy

McDonald, C. M., Abresch, R. T., Carter, G. T., Fowler, W. M., Johnson, E. R., Kilmer, D. D., & Sigford, B. J. (1995) · American Journal of Physical Medicine & Rehabilitation · doi:10.1097/00002060-199509001-00003 · Cited by 1,069

The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy

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. (1991) · Nature · doi:10.1038/352536a0 · Cited by 926

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. (1996) · Nature · doi:10.1038/384349a0 · Cited by 490

Primary structure of dystrophin-related protein

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., et al. (1992) · Nature · doi:10.1038/360591a0 · Cited by 391

Muscular dystrophies and the dystrophin–glycoprotein complex

Straub, V., & Campbell, K. P. (1997) · Current Opinion in Neurology · doi:10.1097/00019052-199704000-00016 · Cited by 383

Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch

Moens, P., Baatsen, P. H. W. W., & Mar�chal, G. (1993) · Journal of Muscle Research and Cell Motility · doi:10.1007/bf00121296 · Cited by 328

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.

Nguyen, T. M., Ellis, J. M., Love, D. R., Davies, K. E., Gatter, K. C., Dickson, G., & Morris, G. E. (1991) · The Journal of cell biology · doi:10.1083/jcb.115.6.1695 · Cited by 266

Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle

Khurana, T. S., Watkins, S. C., Chafey, P., Chelly, J., Tomé, F. M. S., Fardeau, M., Kaplan, J.-C., & Kunkel, L. M. (1991) · Neuromuscular Disorders · doi:10.1016/0960-8966(91)90023-l · Cited by 263

Duchenne muscular dystrophy: Deficiency of dystrophin‐associated proteins in the sarcolemma

Ohlendieck, K., Matsumura, K., Ionasescu, V. V., Towbin, J. A., Bosch, E. P., Weinstein, S. L., Sernett, S. W., & Campbell, K. P. (1993) · Neurology · doi:10.1212/wnl.43.4.795 · Cited by 260

Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice

Deconinck, N., Tinsley, J., Backer, F. D., Fisher, R., Kahn, D., Phelps, S., Davies, K., & Gillis, J.-M. (1997) · Nature Medicine · doi:10.1038/nm1197-1216 · Cited by 250

Dystrophin-deficient mdx muscle fibers are preferentially vulnerable to necrosis induced by experimental lengthening contractions

Weller, B., Karpati, G., & Carpenter, S. (1990) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(90)90005-8 · Cited by 228

Postsynaptic Abnormalities at the Neuromuscular Junctions of Utrophin-deficient Mice

Deconinck, A. E., Potter, A. C., Tinsley, J. M., Wood, S. J., Vater, R., Young, C., Metzinger, L., Vincent, A., Slater, C. R., & Davies, K. E. (1997) · The Journal of Cell Biology · doi:10.1083/jcb.136.4.883 · Cited by 219

Dystrobrevin and dystrophin: An interaction through coiled-coil motifs

Sadoulet-Puccio, H. M., Rajala, M., & Kunkel, L. M. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.23.12413 · Cited by 217

Mammalian alpha 1- and beta 1-syntrophin bind to the alternative splice-prone region of the dystrophin COOH terminus.

Suzuki, A., Yoshida, M., & Ozawa, E. (1995) · The Journal of cell biology · doi:10.1083/jcb.128.3.373 · Cited by 170

Quantitative analysis of the human alpha-skeletal actin gene in transgenic mice.

Brennan, K. J., & Hardeman, E. C. (1993) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)54211-3 · Cited by 167

β-dystrobrevin, a member of the dystrophin-related protein family

Blake, D. J., Nawrotzki, R., Loh, N. Y., Górecki, D. C., & Davies, K. E. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.1.241 · Cited by 153

Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse.

Love, D. R., Morris, G. E., Ellis, J. M., Fairbrother, U. L., Marsden, R. F., Bloomfield, J. F., Edwards, Y. H., Slater, C. P., Parry, D. J., & Davies, K. E. (1991) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.88.8.3243 · Cited by 129

A noninvasive procedure to detect muscle weakness in the mdx mouse

Carlson, C. G., & Makiejus, R. V. (1990) · Muscle & Nerve · doi:10.1002/mus.880130603 · Cited by 74

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., Barnard, E. A., Edwards, Y. H., & Davies, K. E. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.9.3697 · Cited by 71

Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle

Blake, D. J., Weir, A., Newey, S. E., & Davies, K. E. (2002) · Physiological Reviews · doi:10.1152/physrev.00028.2001 · Cited by 1,242

Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy

McGreevy, J. W., Hakim, C. H., McIntosh, M. A., & Duan, D. (2015) · Disease Models & Mechanisms · doi:10.1242/dmm.018424 · Cited by 512

Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy

Allen, D. G., Whitehead, N. P., & Froehner, S. C. (2016) · Physiological Reviews · doi:10.1152/physrev.00007.2015 · Cited by 426

Muscle inactivation of mTOR causes metabolic and dystrophin defects leading to severe myopathy

Risson, V., Mazelin, L., Roceri, M., Sanchez, H., Moncollin, V., Corneloup, C., Richard-Bulteau, H., Vignaud, A., Baas, D., Defour, A., et al. (2009) · Journal of Cell Biology · doi:10.1083/jcb.200903131 · Cited by 381

PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy

Handschin, C., Kobayashi, Y. M., Chin, S., Seale, P., Campbell, K. P., & Spiegelman, B. M. (2007) · Genes & Development · doi:10.1101/gad.1525107 · Cited by 357