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Volumn 11, Issue 26, 2002, Pages 3333-3344

Prevention of pathology in mdx mice by expression of utrophin: Analysis using an inducible transgenic expression system

Author keywords

[No Author keywords available]

Indexed keywords

CALCIUM CHANNEL; CYTOSKELETON PROTEIN; DYSTROPHIN; SARCOGLYCAN; TETRACYCLINE; UTROPHIN;

EID: 12244272395     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/11.26.3333     Document Type: Review
Times cited : (133)

References (43)
  • 1
    • 0036087342 scopus 로고    scopus 로고
    • 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
  • 4
    • 0036796262 scopus 로고    scopus 로고
    • 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
  • 7
    • 0036211418 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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
  • 13
    • 0033994291 scopus 로고    scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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
  • 24
    • 0033382340 scopus 로고    scopus 로고
    • 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
  • 26
    • 0023739851 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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
  • 29
    • 0028414945 scopus 로고
    • 2+ concentrations are not elevated in resting cultured muscle from Duchenne (DMD) patients and in mdx mouse muscle fibres
    • 2+ concentrations are not elevated in resting cultured muscle from Duchenne (DMD) patients and in mdx mouse muscle fibres. Pflügers Archives., 426, 499-505.
    • (1994) Pflügers Archives. , vol.426 , pp. 499-505
    • Pressmar, J.1    Brinkmeier, H.2    Seewald, M.J.3    Naumann, T.4    Rüldel, R.5
  • 30
    • 0033569656 scopus 로고    scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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


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