-
1
-
-
0036087342
-
Function and genetics of dystrophin and dystrophin-related proteins in muscle
-
Blake, D.J., Weir, A., Newey, S.E. and Davies, K.E. (2002) Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol. Rev., 82, 291-329.
-
(2002)
Physiol. Rev.
, vol.82
, pp. 291-329
-
-
Blake, D.J.1
Weir, A.2
Newey, S.E.3
Davies, K.E.4
-
2
-
-
0036127393
-
Modular flexibility of dystrophin: Implications for gene therapy of Duchenne muscular dystrophy
-
Harper, S.Q., Hauser, M.A., DelloRusso, C., Duan, D., Crawford, R.W Phelps, S.F., Harper, H.A., Robinson, A.S., Engelhardt, J.F., Brooks, S.V. et al. (2002) Modular flexibility of dystrophin: Implications for gene therapy of Duchenne muscular dystrophy. Nat. Med., 8, 253-261.
-
(2002)
Nat. Med.
, vol.8
, pp. 253-261
-
-
Harper, S.Q.1
Hauser, M.A.2
DelloRusso, C.3
Duan, D.4
Crawford, R.W.5
Phelps, S.F.6
Harper, H.A.7
Robinson, A.S.8
Engelhardt, J.F.9
Brooks, S.V.10
-
4
-
-
0036796262
-
Gene therapy of muscular dystrophy
-
Chamberlain, J.S. (2002) Gene therapy of muscular dystrophy. Hum. Mol. Genet., 11, 2355- 2362.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 2355-2362
-
-
Chamberlain, J.S.1
-
5
-
-
0035878539
-
Antisense-induced exon skipping restores dystrophin expression in DMD patient derived muscle cells
-
van Deutekom, J.C., Bremmer-Bout, M., Janson, A.A., Ginjaar, I.B., Baas, F, den Dunnen, J.T. and van Ommen, G.J. (2001) Antisense-induced exon skipping restores dystrophin expression in DMD patient derived muscle cells. Hum. Mol. Genet., 10, 1547-1554.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1547-1554
-
-
van Deutekom, J.C.1
Bremmer-Bout, M.2
Janson, A.A.3
Ginjaar, I.B.4
Baas F5
den Dunnen, J.T.6
van Ommen, G.J.7
-
6
-
-
0035793047
-
Antisense-induced exon skipping and synthesis of dystrophin in the mdx mouse
-
Mann, C.J., Honeyman, K., Cheng, A.J., Ly, T, Lloyd, F., Fletcher, S., Morgan, J.E., Partridge, T.A. and Wilton, S.D. (2001) Antisense-induced exon skipping and synthesis of dystrophin in the mdx mouse. Proc. Natl Acad. Sci. USA, 98, 42-47.
-
(2001)
Proc. Natl. Acad. Sci. USA
, vol.98
, pp. 42-47
-
-
Mann, C.J.1
Honeyman, K.2
Cheng, A.J.3
Ly, T.4
Lloyd, F.5
Fletcher, S.6
Morgan, J.E.7
Partridge, T.A.8
Wilton, S.D.9
-
7
-
-
0036211418
-
Dystrophin gene repair in mdx muscle precursor cells in vitro and in vivo mediated by RNA-DNA chimeric oligonucleotides
-
Bertoni, C. and Rando, T.A. (2002) Dystrophin gene repair in mdx muscle precursor cells in vitro and in vivo mediated by RNA-DNA chimeric oligonucleotides. Hum. Gene Ther, 13, 707-718.
-
(2002)
Hum. Gene Ther.
, vol.13
, pp. 707-718
-
-
Bertoni, C.1
Rando, T.A.2
-
8
-
-
0035831649
-
In vivo and in vitro correction of the mdx dystrophin gene nonsense mutation by short-fragment homologous replacement
-
Kapsa, R., Quigley, A., Lynch, G.S., Steeper, K., Kornberg, A.J., Gregorevic, P., Austin, L. and Byrne, E. (2001) In vivo and in vitro correction of the mdx dystrophin gene nonsense mutation by short-fragment homologous replacement. Hum. Gene Ther, 12, 629-642.
-
(2001)
Hum. Gene Ther.
, vol.12
, pp. 629-642
-
-
Kapsa, R.1
Quigley, A.2
Lynch, G.S.3
Steeper, K.4
Kornberg, A.J.5
Gregorevic, P.6
Austin, L.7
Byrne, E.8
-
9
-
-
0029906168
-
Amelioration of the dystrophic phenotype of mdx using a truncated utrophin transgene
-
Tinsley, J.M., Potter, A.C., Phelps, S.R., Fisher, R., Trickett, J.I., Davies, K.E. (1996) Amelioration of the dystrophic phenotype of mdx using a truncated utrophin transgene. Nature, 384, 349-353.
-
(1996)
Nature
, vol.384
, pp. 349-353
-
-
Tinsley, J.M.1
Potter, A.C.2
Phelps, S.R.3
Fisher, R.4
Trickett, J.I.5
Davies, K.E.6
-
10
-
-
0030723508
-
Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice
-
Deconinck, N., Tinsley, J., De Backer, F., Fisher, R., Kahn, D., Phelps, S., Davies, K. and Gillis, J.-M. (1997) Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice. Nat. Med., 3, 1216-1221.
-
(1997)
Nat. Med.
, vol.3
, pp. 1216-1221
-
-
Deconinck, N.1
Tinsley, J.2
De Backer, F.3
Fisher, R.4
Kahn, D.5
Phelps, S.6
Davies, K.7
Gillis, J.-M.8
-
11
-
-
0031727771
-
Expression of full-length utrophin prevents muscular dystrophy in mdx mice
-
Tinsley, J., Deconinck, N., Fisher, R., Kahn, D., Phelps, S., Gillis, J.M. and Davies, K.E. (1998) Expression of full-length utrophin prevents muscular dystrophy in mdx mice. Nat. Med., 4, 1441-1444.
-
(1998)
Nat. Med.
, vol.4
, pp. 1441-1444
-
-
Tinsley, J.1
Deconinck, N.2
Fisher, R.3
Kahn, D.4
Phelps, S.5
Gillis, J.M.6
Davies, K.E.7
-
12
-
-
0034805152
-
Non-toxic ubiquitous over-expression of utrophin in the mdx mouse
-
Fisher, R., Tinsley, J.M., Phelps, S.R., Squire, S.E., Townsend E.R., Martin, J.E. and Davies, K.E. (2001) Non-toxic ubiquitous over-expression of utrophin in the mdx mouse. Neuromusc. Disord., 11, 713-721.
-
(2001)
Neuromusc. Disord.
, vol.11
, pp. 713-721
-
-
Fisher, R.1
Tinsley, J.M.2
Phelps, S.R.3
Squire, S.E.4
Townsend, E.R.5
Martin, J.E.6
Davies, K.E.7
-
13
-
-
0033994291
-
Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenoviral-mediated transfer of a utrophin minigene
-
Wakefield, P.M., Tinsley, J.M., Wood, M.J.A., Gilbert, R., Karpati, G. and Davies, K.E. (2000) Prevention of the dystrophic phenotype in dystrophin/ utrophin-deficient muscle following adenoviral-mediated transfer of a utrophin minigene. Gene Ther, 7, 201-204.
-
(2000)
Gene Ther.
, vol.7
, pp. 201-204
-
-
Wakefield, P.M.1
Tinsley, J.M.2
Wood, M.J.A.3
Gilbert, R.4
Karpati, G.5
Davies, K.E.6
-
14
-
-
0026720075
-
Tight control of gene expression in mammalian cells by tetracycline-responsive promoters
-
Gossen, M. and Bujard, H. (1992) Tight control of gene expression in mammalian cells by tetracycline-responsive promoters. Proc. Natl Acad. Sci. USA, 89, 5547-5551.
-
(1992)
Proc. Natl. Acad. Sci. USA
, vol.89
, pp. 5547-5551
-
-
Gossen, M.1
Bujard, H.2
-
15
-
-
0022767343
-
Transcriptional regulation of the muscle creatine kinase gene and regulated expression in transfected mouse myoblasts
-
Jaynes, J.B., Chamberlain, J.S., Buskin, J.N., Johnson, J.E and Hauschka, S.D. (1986) Transcriptional regulation of the muscle creatine kinase gene and regulated expression in transfected mouse myoblasts. Mol. Cell. Biol., 6, 2855- 2864.
-
(1986)
Mol. Cell Biol.
, vol.6
, pp. 2855-2864
-
-
Jaynes, J.B.1
Chamberlain, J.S.2
Buskin, J.N.3
Johnson, J.E.4
Hauschka, S.D.5
-
16
-
-
0024320433
-
Muscle creatine kinase sequence elements regulating skeletal and cardiac expression in transgenic mice
-
Johnson, J.E., Wold, B.J. and Hauschka, S.D. (1989) Muscle creatine kinase sequence elements regulating skeletal and cardiac expression in transgenic mice. Mol. Cell Biol., 9, 3393-3399.
-
(1989)
Mol. Cell Biol.
, vol.9
, pp. 3393-3399
-
-
Johnson, J.E.1
Wold, B.J.2
Hauschka, S.D.3
-
17
-
-
0027211844
-
Multiple regulatory elements contribute differentially to muscle creatine kinase enhancer activity in skeletal and cardiac muscle
-
Amacher, S.L., Buskin, J.N. and Hauschka, S.D. (1993) Multiple regulatory elements contribute differentially to muscle creatine kinase enhancer activity in skeletal and cardiac muscle. Mol. Cell Biol., 13, 2753-2764.
-
(1993)
Mol. Cell Biol.
, vol.13
, pp. 2753-2764
-
-
Amacher, S.L.1
Buskin, J.N.2
Hauschka, S.D.3
-
18
-
-
0002639380
-
Recherches sur la paralysie musculaire pseudohypertrophique ou paralysie myo-sclérotique
-
5-25, 179-209, 305-321, 421-443
-
Duchenne, G.B.A. (1868) Recherches sur la paralysie musculaire pseudohypertrophique ou paralysie myo-sclérotique. Archives générales de Médecine, 11, 5-25, 179-209, 305-321, 421-443, 552-588.
-
(1868)
Archives Générales De Médecine
, vol.11
, pp. 552-588
-
-
Duchenne, G.B.A.1
-
19
-
-
0023910734
-
The mdx mouse skeletal muscle myopathy: I a histological, morphometric and biochemical investigation
-
Coulton, G.R., Morgan, J.E., Partridge, T.A. and Sloper, J.C. (1988) The mdx mouse skeletal muscle myopathy: I a histological, morphometric and biochemical investigation. Neuropath. Appl. Neurobiol., 14, 53-70.
-
(1988)
Neuropath. Appl. Neurobiol.
, vol.14
, pp. 53-70
-
-
Coulton, G.R.1
Morgan, J.E.2
Partridge, T.A.3
Sloper, J.C.4
-
20
-
-
0025301397
-
A noninvasive procedure to detect muscle weakness in the mdx mouse
-
Carlson, C.G. and Makiejus, R.V. (1990) A noninvasive procedure to detect muscle weakness in the mdx mouse. Mus. Ner., 13, 480-484.
-
(1990)
Mus. Ner.
, vol.13
, pp. 480-484
-
-
Carlson, C.G.1
Makiejus, R.V.2
-
21
-
-
0023832469
-
Fast muscle fibers are preferentially affected in Duchenne muscular dystrophy
-
Webster, C., Silberstein, L., Hays, A.P. and Blau, H.M. (1988) Fast muscle fibers are preferentially affected in Duchenne muscular dystrophy. Cell, 52, 502-513.
-
(1988)
Cell
, vol.52
, pp. 502-513
-
-
Webster, C.1
Silberstein, L.2
Hays, A.P.3
Blau, H.M.4
-
22
-
-
0027248618
-
Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch
-
Moens, P, Baatsen, P.H.W.W. and Maréchal, G. (1993) Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch. J. Musc. Res. Cell Mot., 14, 446-451.
-
(1993)
J. Musc. Res. Cell Mot.
, vol.14
, pp. 446-451
-
-
Moens, P.1
Baatsen, P.H.W.W.2
Maréchal, G.3
-
23
-
-
0027460658
-
Dystrophin protects the sarcolemma from stresses developed during muscle contraction
-
Petrof, B.J., Shrager, J.B., Stedman, H.H., Kelly, A.M. and Sweeney, H.L. (1993) Dystrophin protects the sarcolemma from stresses developed during muscle contraction. Proc. Natl Acad. Sci. USA, 90, 3710-3714.
-
(1993)
Proc. Natl. Acad. Sci. USA
, vol.90
, pp. 3710-3714
-
-
Petrof, B.J.1
Shrager, J.B.2
Stedman, H.H.3
Kelly, A.M.4
Sweeney, H.L.5
-
24
-
-
0033382340
-
Understanding dystrophinopathies: An inventory of the structural and functional consequences of the absence of dystrophin in muscles of the mdx mouse
-
[In Process Citation]
-
Gillis, J.M. (1999) Understanding dystrophinopathies: an inventory of the structural and functional consequences of the absence of dystrophin in muscles of the mdx mouse [In Process Citation]. J. Musc. Res. Cell Mot., 20, 605-625.
-
(1999)
J. Musc. Res. Cell Mot.
, vol.20
, pp. 605-625
-
-
Gillis, J.M.1
-
25
-
-
0026347187
-
Increased calcium influx in dystrophic muscle
-
Turner, P.R., Fong, P., Denetclaw, W.F. and Steinhardt, R.A. (1991) Increased calcium influx in dystrophic muscle. J. Cell Biol., 115, 1701-1712.
-
(1991)
J. Cell Biol.
, vol.115
, pp. 1701-1712
-
-
Turner, P.R.1
Fong, P.2
Denetclaw, W.F.3
Steinhardt, R.A.4
-
26
-
-
0023739851
-
Increased protein degradation results from elevated free calcium levels found in muscle from mdx mice
-
Turner, P.R., Westwood, T., Regen, C.M. and Steinhardt, R.A. (1988) Increased protein degradation results from elevated free calcium levels found in muscle from mdx mice. Nature, 355, 735-738.
-
(1988)
Nature
, vol.355
, pp. 735-738
-
-
Turner, P.R.1
Westwood, T.2
Regen, C.M.3
Steinhardt, R.A.4
-
27
-
-
0027234619
-
Critical evaluation of cytosolic calcium determination in resting muscle fibres from normal and dystrophic (mdx) mice
-
Gailly, P., Boland, B., Himpens, B., Casteels, R. and Gillis, J.M. (1993) Critical evaluation of cytosolic calcium determination in resting muscle fibres from normal and dystrophic (mdx) mice. Cell Calcium, 14, 473-483.
-
(1993)
Cell Calcium
, vol.14
, pp. 473-483
-
-
Gailly, P.1
Boland, B.2
Himpens, B.3
Casteels, R.4
Gillis, J.M.5
-
28
-
-
0027185603
-
Membrane potential, resting calcium and calcium transients in isolated muscle fibres from normal and dystrophic mice
-
Head, S.I. (1993) Membrane potential, resting calcium and calcium transients in isolated muscle fibres from normal and dystrophic mice. J. Physiol., 469, 11-19.
-
(1993)
J. Physiol.
, vol.469
, pp. 11-19
-
-
Head, S.I.1
-
30
-
-
0033569656
-
Intracellular calcium signals measured with indo-1 in isolated skeletal muscle fibres from control and mdx mice
-
Collet, C., Allard, B., Tourneur, Y. and Jacquemond, V. (1999) Intracellular calcium signals measured with indo-1 in isolated skeletal muscle fibres from control and mdx mice. J. Physiol., 520, 417-429.
-
(1999)
J. Physiol.
, vol.520
, pp. 417-429
-
-
Collet, C.1
Allard, B.2
Tourneur, Y.3
Jacquemond, V.4
-
31
-
-
0028052206
-
Myotubes from transgenic mdx mice expressing full-length dystrophin show normal calcium regulation
-
Denetclaw, W.F., Hopf, F.W, Cox, G.A., Chamberlain, J.S. and Steinhardt, R.A. (1994) Myotubes from transgenic mdx mice expressing full-length dystrophin show normal calcium regulation. Mol. Biol. Cell, 5, 1159-1167.
-
(1994)
Mol. Biol. Cell
, vol.5
, pp. 1159-1167
-
-
Denetclaw, W.F.1
Hopf, F.W.2
Cox, G.A.3
Chamberlain, J.S.4
Steinhardt, R.A.5
-
32
-
-
0036686713
-
2+ homeostasis and of survival in collagenase-isolated muscle fibres from normal and mdx mice
-
2+ homeostasis and of survival in collagenase-isolated muscle fibres from normal and mdx mice. J. Physiol., 542, 855-865.
-
(2002)
J. Physiol.
, vol.542
, pp. 855-865
-
-
De Backer, F.1
Vandebrouck, C.2
Gailly, P.3
Gillis, J.M.4
-
33
-
-
0035999982
-
Utrophin binds laterally along actin filaments and can couple costameric actin with sarcolemma when overexpressed in dystrophin-deficient muscle
-
Rybakova, I.N., Patel, J.R., Davies, K.E. Yurchenco, P.D. and Ervasti, J.M. (2002) Utrophin binds laterally along actin filaments and can couple costameric actin with sarcolemma when overexpressed in dystrophin-deficient muscle. Mol. Biol. Cell., 13, 1512-1221.
-
(2002)
Mol. Biol. Cell
, vol.13
, pp. 1221-1512
-
-
Rybakova, I.N.1
Patel, J.R.2
Davies, K.E.3
Yurchenco, P.D.4
Ervasti, J.M.5
-
34
-
-
0034641893
-
mdx mice inducibly expressing dystrophin provide insights into the potential of gene therapy for Duchenne muscular dystrophy
-
Ahmad, A., Brinson, M., Hodges, B.L., Chamberlain, J.S. and Amalfitano, A. (2000) mdx mice inducibly expressing dystrophin provide insights into the potential of gene therapy for Duchenne muscular dystrophy. Hum. Mol. Genet., 9, 2507-2515.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 2507-2515
-
-
Ahmad, A.1
Brinson, M.2
Hodges, B.L.3
Chamberlain, J.S.4
Amalfitano, A.5
-
35
-
-
0025222219
-
Increased activity of calcium leak channels in myotubes of Duchenne human and mdx mouse origin
-
Fong, P., Turner, P.R., Denetclaw, W.F. and Steinhardt, R.A. (1990) Increased activity of calcium leak channels in myotubes of Duchenne human and mdx mouse origin. Science, 250, 673-676.
-
(1990)
Science
, vol.250
, pp. 673-676
-
-
Fong, P.1
Turner, P.R.2
Denetclaw, W.F.3
Steinhardt, R.A.4
-
36
-
-
0027281140
-
Proteolysis results in altered leak channel kinetics and elevated free calcium in mdx muscle
-
Turner, P.R., Schultz, R., Ganguly, B. and Steinhardt, R.A. (1993) Proteolysis results in altered leak channel kinetics and elevated free calcium in mdx muscle. J. Memb. Biol., 133, 243-251.
-
(1993)
J. Memb. Biol.
, vol.133
, pp. 243-251
-
-
Turner, P.R.1
Schultz, R.2
Ganguly, B.3
Steinhardt, R.A.4
-
37
-
-
0028564748
-
Mechanosensitive ion channels in skeletal muscle from normal and dystrophic mice
-
Franco-Obregon, A. Jr. and Lansman, J.B. (1994) Mechanosensitive ion channels in skeletal muscle from normal and dystrophic mice. J. Physiol., 1, 299-309.
-
(1994)
J. Physiol.
, vol.1
, pp. 299-309
-
-
Franco-Obregon A., Jr.1
Lansman, J.B.2
-
38
-
-
0031670642
-
Highly controlled gene expression using combinations of a tissue-specific promoter, recombinant adenovirus and a tetracycline-regulatable transcription factor
-
Ghersa, P., Gobert, R.P, Sattonnet-Roche, P., Richards, C.A., Merlo Pich, E. and Hooft van Huijsduijnen, R. (1998) Highly controlled gene expression using combinations of a tissue-specific promoter, recombinant adenovirus and a tetracycline-regulatable transcription factor. Gene Ther., 5, 1213-1220.
-
(1998)
Gene Ther.
, vol.5
, pp. 1213-1220
-
-
Ghersa, P.1
Gobert, R.P.2
Sattonnet-Roche, P.3
Richards, C.A.4
Merlo Pich, E.5
Hooft van Huijsduijnen, R.6
-
39
-
-
0030566806
-
Cloning and expression of full length mouse utrophin: The differential association of utrophin and dystrophin with AChR clusters
-
Guo, W.X., Nichol, M. and Merlie, J.P. (1996) Cloning and expression of full length mouse utrophin: the differential association of utrophin and dystrophin with AChR clusters. FEBS Lett., 398, 259-264.
-
(1996)
FEBS Lett.
, vol.398
, pp. 259-264
-
-
Guo, W.X.1
Nichol, M.2
Merlie, J.P.3
-
41
-
-
0029918603
-
Functional protection of dystrophic mouse (mdx) muscles after adenovirus-mediated transfer of a dystrophin minigene
-
Deconinck, N., Ragot, T, Maréchal, G., Pérricaudet, M. and Gillis, J.M. (1996) Functional protection of dystrophic mouse (mdx) muscles after adenovirus-mediated transfer of a dystrophin minigene. Proc. Natl Acad. Sci. USA, 93, 3570-3574.
-
(1996)
Proc. Natl. Acad. Sci. USA
, vol.93
, pp. 3570-3574
-
-
Deconinck, N.1
Ragot, T.2
Maréchal, G.3
Pérricaudet, M.4
Gillis, J.M.5
-
42
-
-
0019441262
-
Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches
-
Hamill, O.P., Marty, A., Neher, E., Sakmann, B. and Sigworth, F.J. (1981) Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches. Pflügers Archives, 391, 85-100.
-
(1981)
Pflügers Archives
, vol.391
, pp. 85-100
-
-
Hamill, O.P.1
Marty, A.2
Neher, E.3
Sakmann, B.4
Sigworth, F.J.5
-
43
-
-
0033863129
-
Increased activity of calcium leak channels caused by proteolysis near sarcolemmal ruptures
-
McCarter, G.C. and Steinhardt, R.A. (2000) Increased activity of calcium leak channels caused by proteolysis near sarcolemmal ruptures. J. Memb. Biol., 176, 169-174.
-
(2000)
J. Memb. Biol.
, vol.176
, pp. 169-174
-
-
McCarter, G.C.1
Steinhardt, R.A.2
|