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Science 30 June 1989: Vol. 244. no. 4912, pp. 1578 - 1580 DOI: 10.1126/science.2662404
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Articles
Science, Vol 244, Issue 4912, 1578-1580
Copyright © 1989 by American Association for the Advancement of Science
The molecular basis of muscular dystrophy in the mdx mouse: a point mutation
P Sicinski,
Y Geng,
AS Ryder-Cook,
EA Barnard,
MG Darlison,
and
PJ Barnard
Medical Research Unit, MRC Centre, Cambridge, United Kingdom.
The mdx mouse is an X-linked myopathic mutant, an animal model for human Duchenne muscular dystrophy. In both mouse and man the mutations lie within the dystrophin gene, but the phenotypic differences of the disease in the two species confer much interest on the molecular basis of the mdx mutation. The complementary DNA for mouse dystrophin has been cloned, and the sequence has been used in the polymerase chain reaction to amplify normal and mdx dystrophin transcripts in the area of the mdx mutation. Sequence analysis of the amplification products showed that the mdx mouse has a single base substitution within an exon, which causes premature termination of the polypeptide chain.
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| Full Text »
| PDF »
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| Full Text »
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| Full Text »
| PDF »
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| Full Text »
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| Abstract »
| Full Text »
| PDF »
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| Abstract »
| Full Text »
| PDF »
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| Abstract »
| Full Text »
| PDF »
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| Abstract »
| Full Text »
| PDF »
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| Abstract »
| Full Text »
| PDF »
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| Abstract »
| Full Text »
| PDF »
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- M. J. Allikian, A. A. Hack, S. Mewborn, U. Mayer, and E. M. McNally (2004)
J. Cell Sci.
117, 3821-3830
| Abstract »
| Full Text »
| PDF »
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- E. J. Mientjes, R. Willemsen, L. L. Kirkpatrick, I. M. Nieuwenhuizen, M. Hoogeveen-Westerveld, M. Verweij, S. Reis, B. Bardoni, A. T. Hoogeveen, B. A. Oostra, et al. (2004)
Hum. Mol. Genet.
13, 1291-1302
| Abstract »
| Full Text »
| PDF »
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| Abstract »
| Full Text »
| PDF »
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350, 2682-2688
| Full Text »
| PDF »
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- Y. Nakayama, N. Nara, Y. Kawakita, Y. Takeshima, M. Arakawa, M. Katoh, S. Morita, K. Iwatsuki, K. Tanaka, S. Okamoto, et al. (2004)
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| Abstract »
| Full Text »
| PDF »
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- K. A. Lapidos, R. Kakkar, and E. M. McNally (2004)
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| Abstract »
| Full Text »
| PDF »
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- F. Rivier, O. Alkan, A. F. Flint, K. Muskiewicz, P. D. Allen, P. Leboulch, and E. Gussoni (2004)
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| Abstract »
| Full Text »
| PDF »
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- M. Ishikawa-Sakurai, M. Yoshida, M. Imamura, K. E. Davies, and E. Ozawa (2004)
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| Abstract »
| Full Text »
| PDF »
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- E. Bachrach, S. Li, A. L. Perez, J. Schienda, K. Liadaki, J. Volinski, A. Flint, J. Chamberlain, and L. M. Kunkel (2004)
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101, 3581-3586
| Abstract »
| Full Text »
| PDF »
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- S. B. P. CHARGE and M. A. RUDNICKI (2004)
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84, 209-238
| Abstract »
| Full Text »
| PDF »
- Dystrophin is required for the formation of stable muscle attachments in the zebrafish embryo.
- D. I. Bassett, R. J. Bryson-Richardson, D. F. Daggett, P. Gautier, D. G. Keenan, and P. D. Currie (2003)
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130, 5851-5860
| Abstract »
| Full Text »
| PDF »
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- M. Arakawa, M. Shiozuka, Y. Nakayama, T. Hara, M. Hamada, S.'i. Kondo, D. Ikeda, Y. Takahashi, R. Sawa, Y. Nonomura, et al. (2003)
J. Biochem.
134, 751-758
| Abstract »
| Full Text »
| PDF »
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- G. Bonuccelli, F. Sotgia, W. Schubert, D. S. Park, P. G. Frank, S. E. Woodman, L. Insabato, M. Cammer, C. Minetti, and M. P. Lisanti (2003)
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163, 1663-1675
| Abstract »
| Full Text »
| PDF »
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- D. R. Plant and G. S. Lynch (2003)
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| Abstract »
| Full Text »
| PDF »
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- F. Montanaro, K. Liadaki, J. Volinski, A. Flint, and L. M. Kunkel (2003)
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100, 9336-9341
| Abstract »
| Full Text »
| PDF »
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- C. De Bari, F. Dell'Accio, F. Vandenabeele, J. R. Vermeesch, J.-M. Raymackers, and F. P. Luyten (2003)
J. Cell Biol.
160, 909-918
| Abstract »
| Full Text »
| PDF »
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- R. Nawrotzki, M. Willem, N. Miosge, H. Brinkmeier, and U. Mayer (2003)
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12, 483-495
| Abstract »
| Full Text »
| PDF »
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- A. KUMAR and A. M. BORIEK (2003)
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17, 386-396
| Abstract »
| Full Text »
| PDF »
- Functional correction of adult mdx mouse muscle using gutted adenoviral vectors expressing full-length dystrophin.
- C. DelloRusso, J. M. Scott, D. Hartigan-O'Connor, G. Salvatori, C. Barjot, A. S. Robinson, R. W. Crawford, S. V. Brooks, and J. S. Chamberlain (2002)
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99, 12979-12984
| Abstract »
| Full Text »
| PDF »
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- M. T. Wheeler, S. Zarnegar, and E. M. McNally (2002)
Hum. Mol. Genet.
11, 2147-2154
| Abstract »
| Full Text »
| PDF »
- Global/temporal gene expression in diaphragm and hindlimb muscles of dystrophin-deficient (mdx) mice.
- K. Rouger, M. Le Cunff, M. Steenman, M.-C. Potier, N. Gibelin, C. A. Dechesne, and J. J. Leger (2002)
Am J Physiol Cell Physiol
283, C773-C784
| Abstract »
| Full Text »
| PDF »
- Functional characteristics of dystrophic skeletal muscle: insights from animal models.
- J. F. Watchko, T. L. O'Day, and E. P. Hoffman (2002)
J Appl Physiol
93, 407-417
| Abstract »
| Full Text »
| PDF »
- Long-term study of Ca2+ homeostasis and of survival in collagenase-isolated muscle fibres from normal and mdx mice.
- F De Backer, C Vandebrouck, P Gailly, and J M Gillis (2002)
J. Physiol.
542, 855-865
| Abstract »
| Full Text »
| PDF »
- Green tea extract decreases muscle necrosis in mdx mice and protects against reactive oxygen species.
- T. M Buetler, M. Renard, E. A Offord, H. Schneider, and U. T Ruegg (2002)
Am. J. Clinical Nutrition
75, 749-753
| Abstract »
| Full Text »
| PDF »
- Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle.
- D. J. Blake, A. Weir, S. E. Newey, and K. E. Davies (2002)
Physiol Rev
82, 291-329
| Abstract »
| Full Text »
| PDF »
- Muscle-specific expression of insulin-like growth factor I counters muscle decline in mdx mice.
- E. R. Barton, L. Morris, A. Musaro, N. Rosenthal, and H. L. Sweeney (2002)
J. Cell Biol.
157, 137-148
| Abstract »
| Full Text »
| PDF »
- The Sarcoglycan Complex in Striated and Vascular Smooth Muscle.
- M.T. WHEELER, M.J. ALLIKIAN, A. HEYDEMANN, and E.M. MCNALLY (2002)
Cold Spring Harb Symp Quant Biol
67, 389-398
| Abstract »
| PDF »
- Insulin-like Growth Factor Isoforms in Skeletal Muscle Aging, Regeneration, and Disease.
- N. WINN, A. PAUL, A. MUSARO, and N. ROSENTHAL (2002)
Cold Spring Harb Symp Quant Biol
67, 507-518
| Abstract »
| PDF »
- Suppression of revertant fibers in mdx mice by expression of a functional dystrophin.
- G. E. Crawford, Q. L. Lu, T. A. Partridge, and J. S. Chamberlain (2001)
Hum. Mol. Genet.
10, 2745-2750
| Abstract »
| Full Text »
| PDF »
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- F. Liu, K. W. Liang, and L. Huang (2001)
Mol. Interv.
1, 168-172
| Abstract »
| Full Text »
| PDF »
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- Y. Kamogawa, S. Biro, M. Maeda, M. Setoguchi, T. Hirakawa, H. Yoshida, and C. Tei (2001)
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| Abstract »
| Full Text »
| PDF »
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- D. J. Burkin, G. Q. Wallace, K. J. Nicol, D. J. Kaufman, and S. J. Kaufman (2001)
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152, 1207-1218
| Abstract »
| Full Text »
| PDF »
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- Y. Torrente, J.-P Tremblay, F. Pisati, M. Belicchi, B. Rossi, M. Sironi, F. Fortunato, M. El Fahime, M. G. D'Angelo, N. J. Caron, et al. (2001)
J. Cell Biol.
152, 335-348
| Abstract »
| Full Text »
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- V. Allamand and K. P. Campbell (2000)
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| Abstract »
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- G. S. Lynch, J. A. Rafael, J. S. Chamberlain, and J. A. Faulkner (2000)
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| Abstract »
| Full Text »
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- G. Felisari, F. M. Boneschi, A. Bardoni, M. Sironi, G. P. Comi, M. Robotti, A. C. Turconi, M. Lai, G. Corrao, and N. Bresolin (2000)
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| Abstract »
| Full Text »
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- Y. Torrente, M.G. D'Angelo, Z. Li, R. Del Bo, S. Corti, M. Mericskay, A. DeLiso, A. Fassati, D. Paulin, G.P. Comi, et al. (2000)
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9, 1843-1852
| Abstract »
| Full Text »
| PDF »
- Severe Mechanical Dysfunction in Pharyngeal Muscle from Adult mdx Mice.
- P. ATTAL, F. LAMBERT, S. MARCHAND-ADAM, S. BOBIN, J.-C. POURNY, D. CHEMLA, Y. LECARPENTIER, and C. COIRAULT (2000)
Am. J. Respir. Crit. Care Med.
162, 278-281
| Abstract »
| Full Text »
- Distinct Patterns of Dystrophin Organization in Myocyte Sarcolemma and Transverse Tubules of Normal and Diseased Human Myocardium.
- R. R. Kaprielian, S. Stevenson, S. M. Rothery, M. J. Cullen, and N. J. Severs (2000)
Circulation
101, 2586-2594
| Abstract »
| Full Text »
| PDF »
- Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure.
- J. A. Rafael, E. R. Townsend, S. E. Squire, A. C. Potter, J. S. Chamberlain, and K. E. Davies (2000)
Hum. Mol. Genet.
9, 1357-1367
| Abstract »
| Full Text »
| PDF »
- Rescue of dystrophin expression in mdx mouse muscle by RNA/DNA oligonucleotides.
- T. A. Rando, M.-H. Disatnik, and L. Z.-H. Zhou (2000)
PNAS
97, 5363-5368
| Abstract »
| Full Text »
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- Massive Idiosyncratic Exon Skipping Corrects the Nonsense Mutation in Dystrophic Mouse Muscle and Produces Functional Revertant Fibers by Clonal Expansion.
- Q.L. Lu, G.E. Morris, S.D. Wilton, T. Ly, O.V. Artem'yeva, P. Strong, and T.A. Partridge (2000)
J. Cell Biol.
148, 985-996
| Abstract »
| Full Text »
| PDF »
- Myoferlin, a candidate gene and potential modifier of muscular dystrophy.
- D. B. Davis, A. J. Delmonte, C. T. Ly, and E. M. McNally (2000)
Hum. Mol. Genet.
9, 217-226
| Abstract »
| Full Text »
| PDF »
- Muscle degeneration without mechanical injury in sarcoglycan deficiency.
- A. A. Hack, L. Cordier, D. I. Shoturma, M. Y. Lam, H. L. Sweeney, and E. M. McNally (1999)
PNAS
96, 10723-10728
| Abstract »
| Full Text »
| PDF »
- The Vitamin D Receptor and the Syndrome of Hereditary 1,25-Dihydroxyvitamin D-Resistant Rickets.
- P. J. Malloy, J. W. Pike, and D. Feldman (1999)
Endocr. Rev.
20, 156-188
| Abstract »
| Full Text »
- Hindlimb immobilization applied to 21-day-old mdx mice prevents the occurrence of muscle degeneration.
- A. Mokhtarian, J. P. Lefaucheur, P. C. Even, and A. Sebille (1999)
J Appl Physiol
86, 924-931
| Abstract »
| Full Text »
| PDF »
- Severe cardiomyopathy in mice lacking dystrophin and MyoD.
- L. A. Megeney, B. Kablar, R. L. S. Perry, C. Ying, L. May, and M. A. Rudnicki (1999)
PNAS
96, 220-225
| Abstract »
| Full Text »
| PDF »
- Development of Approaches to Improve Cell Survival in Myoblast Transfer Therapy.
- Z. Qu, L. Balkir, J. C.T. van Deutekom, P. D. Robbins, R. Pruchnic, and J. Huard (1998)
J. Cell Biol.
142, 1257-1267
| Abstract »
| Full Text »
| PDF »
- {gamma}-Sarcoglycan Deficiency Leads to Muscle Membrane Defects and Apoptosis Independent of Dystrophin.
- A. A. Hack, C. T. Ly, F. Jiang, C. J. Clendenin, K. S. Sigrist, R. L. Wollmann, and E. M. McNally (1998)
J. Cell Biol.
142, 1279-1287
| Abstract »
| Full Text »
| PDF »
- Contractile function and low-intensity exercise effects of old dystrophic (mdx) mice.
- A. Hayes and D. A. Williams (1998)
Am J Physiol Cell Physiol
274, C1138-C1144
| Abstract »
| Full Text »
| PDF »
- {beta}-Spectrin Is Colocalized with Both Voltage-gated Sodium Channels and AnkyrinG at the Adult Rat Neuromuscular Junction.
- S.J. Wood and C.R. Slater (1998)
J. Cell Biol.
140, 675-684
| Abstract »
| Full Text »
| PDF »
- Acetylcholine Receptors in Innervated Muscles of Dystrophic mdx Mice Degrade as after Denervation.
- R. Xu and M. M. Salpeter (1997)
J. Neurosci.
17, 8194-8200
| Abstract »
| Full Text »
| PDF »
- Postsynaptic Abnormalities at the Neuromuscular Junctions of Utrophin-deficient Mice.
- A. E. Deconinck, A. C. Potter, J. M. Tinsley, S. J. Wood, R. Vater, C. Young, L. Metzinger, A. Vincent, C. R. Slater, and K. E. Davies (1997)
J. Cell Biol.
136, 883-894
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| Full Text »
| PDF »
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