메뉴 건너뛰기




Volumn 84, Issue , 2008, Pages 431-453

Chapter 9 The Value of Mammalian Models for Duchenne Muscular Dystrophy in Developing Therapeutic Strategies

Author keywords

[No Author keywords available]

Indexed keywords

DYSTROPHIN; UTROPHIN;

EID: 58749101371     PISSN: 00702153     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S0070-2153(08)00609-1     Document Type: Review
Times cited : (106)

References (163)
  • 2
    • 0034468908 scopus 로고    scopus 로고
    • How calcium influx through calcium leak channels is responsible for the elevated levels of calcium-dependent proteolysis in dystrophic myotubes
    • Alderton J.M., and Steinhardt R.A. How calcium influx through calcium leak channels is responsible for the elevated levels of calcium-dependent proteolysis in dystrophic myotubes. Trends Cardiovasc. Med. 10 (2000) 268-272
    • (2000) Trends Cardiovasc. Med. , vol.10 , pp. 268-272
    • Alderton, J.M.1    Steinhardt, R.A.2
  • 3
    • 32244443828 scopus 로고    scopus 로고
    • Systemic delivery of morpholino oligonucleotide restores dystrophin expression bodywide and improves dystrophic pathology
    • Alter J., Lou F., Rabinowitz A., Yin H., Rosenfeld J., Wilton S.D., Partridge T.A., and Lu Q.L. Systemic delivery of morpholino oligonucleotide restores dystrophin expression bodywide and improves dystrophic pathology. Nat. Med. 12 (2006) 175-177
    • (2006) Nat. Med. , vol.12 , pp. 175-177
    • Alter, J.1    Lou, F.2    Rabinowitz, A.3    Yin, H.4    Rosenfeld, J.5    Wilton, S.D.6    Partridge, T.A.7    Lu, Q.L.8
  • 4
    • 0032561199 scopus 로고    scopus 로고
    • A cluster of basic repeats in the dystrophin rod domain binds F-actin through an electrostatic interaction
    • Amann K.J., Renley B.A., and Ervasti J.M. A cluster of basic repeats in the dystrophin rod domain binds F-actin through an electrostatic interaction. J. Biol. Chem. 273 (1998) 28419-28423
    • (1998) J. Biol. Chem. , vol.273 , pp. 28419-28423
    • Amann, K.J.1    Renley, B.A.2    Ervasti, J.M.3
  • 5
    • 3042629357 scopus 로고    scopus 로고
    • The postsynaptic submembrane machinery at the neuromuscular junction: Requirement for rapsyn and the utrophin/dystrophin-associated complex
    • Banks G.B., Fuhrer C., Adams M.E., and Froehner S.C. The postsynaptic submembrane machinery at the neuromuscular junction: Requirement for rapsyn and the utrophin/dystrophin-associated complex. J. Neurocytol. 32 (2003) 709-726
    • (2003) J. Neurocytol. , vol.32 , pp. 709-726
    • Banks, G.B.1    Fuhrer, C.2    Adams, M.E.3    Froehner, S.C.4
  • 6
    • 34548419653 scopus 로고    scopus 로고
    • Functional capacity of dystrophins carrying deletions in the N-terminal actin-binding domain
    • Banks G.B., Gregorevic P., Allen J.M., Finn E.E., and Chamberlain J.S. Functional capacity of dystrophins carrying deletions in the N-terminal actin-binding domain. Hum. Mol. Genet. 16 (2007) 2105-2113
    • (2007) Hum. Mol. Genet. , vol.16 , pp. 2105-2113
    • Banks, G.B.1    Gregorevic, P.2    Allen, J.M.3    Finn, E.E.4    Chamberlain, J.S.5
  • 7
    • 0024580404 scopus 로고
    • Molecular and clinical correlations of deletions leading to Duchenne and Becker muscular dystrophies
    • Baumbach L.L., Chamberlain J.S., Ward P.A., Farwell N.J., and Caskey C.T. Molecular and clinical correlations of deletions leading to Duchenne and Becker muscular dystrophies. Neurology 39 (1989) 465-474
    • (1989) Neurology , vol.39 , pp. 465-474
    • Baumbach, L.L.1    Chamberlain, J.S.2    Ward, P.A.3    Farwell, N.J.4    Caskey, C.T.5
  • 8
    • 0033594119 scopus 로고    scopus 로고
    • Dynamics of myoblast transplantation reveal a discrete minority of precursors with stem cell-like properties as the myogenic source
    • Beauchamp J.R., Morgan J.E., Pagel C.N., and Partridge T.A. Dynamics of myoblast transplantation reveal a discrete minority of precursors with stem cell-like properties as the myogenic source. J. Cell Biol. 144 (1999) 1113-1122
    • (1999) J. Cell Biol. , vol.144 , pp. 1113-1122
    • Beauchamp, J.R.1    Morgan, J.E.2    Pagel, C.N.3    Partridge, T.A.4
  • 10
    • 0014353260 scopus 로고
    • Histopathological changes in Duchenne muscular dystrophy
    • Bell C.D., and Conen P.E. Histopathological changes in Duchenne muscular dystrophy. J. Neurol. Sci. 7 (1968) 529-544
    • (1968) J. Neurol. Sci. , vol.7 , pp. 529-544
    • Bell, C.D.1    Conen, P.E.2
  • 12
    • 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. Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol. Rev. 82 (2002) 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
  • 13
    • 0037391465 scopus 로고    scopus 로고
    • Lateral force transmission across costameres in skeletal muscle
    • Bloch R.J., and Gonzalez-Serratos H. Lateral force transmission across costameres in skeletal muscle. Exerc. Sport Sci. Rev. 31 (2003) 73-78
    • (2003) Exerc. Sport Sci. Rev. , vol.31 , pp. 73-78
    • Bloch, R.J.1    Gonzalez-Serratos, H.2
  • 15
    • 50649083054 scopus 로고    scopus 로고
    • AAV-9 micro-dystrophin gene therapy ameliorates electrocardiographic abnormalities in mdx mice
    • Bostick B., Yue Y., Lai Y., Long C., Li D., and Dongsheng D. AAV-9 micro-dystrophin gene therapy ameliorates electrocardiographic abnormalities in mdx mice. Hum. Gene Ther (2008)
    • (2008) Hum. Gene Ther
    • Bostick, B.1    Yue, Y.2    Lai, Y.3    Long, C.4    Li, D.5    Dongsheng, D.6
  • 16
    • 36248973631 scopus 로고    scopus 로고
    • Intrinsic changes and extrinsic influences of myogenic stem cell function during aging
    • Brack A.S., and Rando T.A. Intrinsic changes and extrinsic influences of myogenic stem cell function during aging. Stem. Cell Rev. 3 (2007) 226-237
    • (2007) Stem. Cell Rev. , vol.3 , pp. 226-237
    • Brack, A.S.1    Rando, T.A.2
  • 17
    • 0017886146 scopus 로고
    • Studies of sarcolemmal integrity in myopathic muscle
    • Bradley W.G., and Fulthorpe J.J. Studies of sarcolemmal integrity in myopathic muscle. Neurology 28 (1978) 670-677
    • (1978) Neurology , vol.28 , pp. 670-677
    • Bradley, W.G.1    Fulthorpe, J.J.2
  • 18
    • 0029149471 scopus 로고
    • Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy
    • Brenman J.E., Chao D.S., Xia H., Aldape K., and Bredt D.S. Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy. Cell 82 (1995) 743-752
    • (1995) Cell , vol.82 , pp. 743-752
    • Brenman, J.E.1    Chao, D.S.2    Xia, H.3    Aldape, K.4    Bredt, D.S.5
  • 19
    • 37549042455 scopus 로고    scopus 로고
    • Stem cell treatment of dystrophic dogs
    • discussion E23-E25
    • Bretag A.H. Stem cell treatment of dystrophic dogs. Nature 450 (2007) E23 discussion E23-E25
    • (2007) Nature , vol.450
    • Bretag, A.H.1
  • 20
    • 0031906615 scopus 로고    scopus 로고
    • Rapid recovery following contraction-induced injury to in situ skeletal muscles in mdx mice
    • Brooks S.V. Rapid recovery following contraction-induced injury to in situ skeletal muscles in mdx mice. J. Muscle Res. Cell Motil. 19 (1998) 179-187
    • (1998) J. Muscle Res. Cell Motil. , vol.19 , pp. 179-187
    • Brooks, S.V.1
  • 22
    • 0033598694 scopus 로고    scopus 로고
    • A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy
    • Burton E.A., Tinsley J.M., Holzfeind P.J., Rodrigues N.R., and Davies K.E. A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy. Proc. Natl. Acad. Sci. USA 96 (1999) 14025-14030
    • (1999) Proc. Natl. Acad. Sci. USA , vol.96 , pp. 14025-14030
    • Burton, E.A.1    Tinsley, J.M.2    Holzfeind, P.J.3    Rodrigues, N.R.4    Davies, K.E.5
  • 23
    • 0034956422 scopus 로고    scopus 로고
    • Adult dystrophic (mdx) endplates exhibit reduced quantal size and enhanced quantal variation
    • Carlson C.G., and Roshek D.M. Adult dystrophic (mdx) endplates exhibit reduced quantal size and enhanced quantal variation. Pflugers Arch. 442 (2001) 369-375
    • (2001) Pflugers Arch. , vol.442 , pp. 369-375
    • Carlson, C.G.1    Roshek, D.M.2
  • 24
    • 47949097215 scopus 로고    scopus 로고
    • Imbalance between pSmad3 and Notch induces CDK inhibitors in old muscle stem cells
    • Carlson M.E., Hsu M., and Conboy I.M. Imbalance between pSmad3 and Notch induces CDK inhibitors in old muscle stem cells. Nature (2008)
    • (2008) Nature
    • Carlson, M.E.1    Hsu, M.2    Conboy, I.M.3
  • 27
    • 0024245082 scopus 로고
    • Deletion screening of the Duchenne muscular dystrophy locus via multiplex DNA amplification
    • Chamberlain J.S., Gibbs R.A., Ranier J.E., Nguyen P.N., and Caskey C.T. Deletion screening of the Duchenne muscular dystrophy locus via multiplex DNA amplification. Nucleic Acids Res. 16 (1988) 11141-11156
    • (1988) Nucleic Acids Res. , vol.16 , pp. 11141-11156
    • Chamberlain, J.S.1    Gibbs, R.A.2    Ranier, J.E.3    Nguyen, P.N.4    Caskey, C.T.5
  • 29
    • 34347374860 scopus 로고    scopus 로고
    • Dystrophin-deficient mdx mice display a reduced life span and are susceptible to spontaneous rhabdomyosarcoma
    • Chamberlain J.S., Metzger J., Reyes M., Townsend D., and Faulkner J.A. Dystrophin-deficient mdx mice display a reduced life span and are susceptible to spontaneous rhabdomyosarcoma. FASEB J. 21 (2007) 2195-2204
    • (2007) FASEB J. , vol.21 , pp. 2195-2204
    • Chamberlain, J.S.1    Metzger, J.2    Reyes, M.3    Townsend, D.4    Faulkner, J.A.5
  • 31
    • 0025021669 scopus 로고
    • Dystrophin gene transcribed from different promoters in neuronal and glial cells
    • Chelly J., Hamard G., Koulakoff A., Kaplan J.C., Kahn A., and Berwald-Netter Y. Dystrophin gene transcribed from different promoters in neuronal and glial cells. Nature 344 (1990) 64-65
    • (1990) Nature , vol.344 , pp. 64-65
    • Chelly, J.1    Hamard, G.2    Koulakoff, A.3    Kaplan, J.C.4    Kahn, A.5    Berwald-Netter, Y.6
  • 32
    • 5644253274 scopus 로고    scopus 로고
    • Tissue Doppler assessment of diastolic and systolic alterations of radial and longitudinal left ventricular motions in Golden Retrievers during the preclinical phase of cardiomyopathy associated with muscular dystrophy
    • Chetboul V., Carlos C., Blot S., Thibaud J.L., Escriou C., Tissier R., Retortillo J.L., and Pouchelon J.L. Tissue Doppler assessment of diastolic and systolic alterations of radial and longitudinal left ventricular motions in Golden Retrievers during the preclinical phase of cardiomyopathy associated with muscular dystrophy. Am. J. Vet. Res. 65 (2004) 1335-1341
    • (2004) Am. J. Vet. Res. , vol.65 , pp. 1335-1341
    • Chetboul, V.1    Carlos, C.2    Blot, S.3    Thibaud, J.L.4    Escriou, C.5    Tissier, R.6    Retortillo, J.L.7    Pouchelon, J.L.8
  • 36
    • 22744438723 scopus 로고    scopus 로고
    • Stem cell function, self-renewal, and behavioral heterogeneity of cells from the adult muscle satellite cell niche
    • Collins C.A., Olsen I., Zammit P.S., Heslop L., Petrie A., Partridge T.A., and Morgan J.E. Stem cell function, self-renewal, and behavioral heterogeneity of cells from the adult muscle satellite cell niche. Cell 122 (2005) 289-301
    • (2005) Cell , vol.122 , pp. 289-301
    • Collins, C.A.1    Olsen, I.2    Zammit, P.S.3    Heslop, L.4    Petrie, A.5    Partridge, T.A.6    Morgan, J.E.7
  • 37
    • 0344827208 scopus 로고    scopus 로고
    • Notch-mediated restoration of regenerative potential to aged muscle
    • Conboy I.M., Conboy M.J., Smythe G.M., and Rando T.A. Notch-mediated restoration of regenerative potential to aged muscle. Science 302 (2003) 1575-1577
    • (2003) Science , vol.302 , pp. 1575-1577
    • Conboy, I.M.1    Conboy, M.J.2    Smythe, G.M.3    Rando, T.A.4
  • 38
    • 13944261231 scopus 로고    scopus 로고
    • Rejuvenation of aged progenitor cells by exposure to a young systemic environment
    • Conboy I.M., Conboy M.J., Wagers A.J., Girma E.R., Weissman I.L., and Rando T.A. Rejuvenation of aged progenitor cells by exposure to a young systemic environment. Nature 433 (2005) 760-764
    • (2005) Nature , vol.433 , pp. 760-764
    • Conboy, I.M.1    Conboy, M.J.2    Wagers, A.J.3    Girma, E.R.4    Weissman, I.L.5    Rando, T.A.6
  • 40
    • 0025341845 scopus 로고
    • Mosaic expression of dystrophin in carriers of canine X-linked muscular dystrophy
    • Cooper B.J., Gallagher E.A., Smith C.A., Valentine B.A., and Winand N.J. Mosaic expression of dystrophin in carriers of canine X-linked muscular dystrophy. Lab. Invest. 62 (1990) 171-178
    • (1990) Lab. Invest. , vol.62 , pp. 171-178
    • Cooper, B.J.1    Gallagher, E.A.2    Smith, C.A.3    Valentine, B.A.4    Winand, N.J.5
  • 41
    • 0027273835 scopus 로고
    • New mdx mutation disrupts expression of muscle and nonmuscle isoforms of dystrophin
    • Cox G.A., Phelps S.F., Chapman V.M., and Chamberlain J.S. New mdx mutation disrupts expression of muscle and nonmuscle isoforms of dystrophin. Nat. Genet. 4 (1993) 87-93
    • (1993) Nat. Genet. , vol.4 , pp. 87-93
    • Cox, G.A.1    Phelps, S.F.2    Chapman, V.M.3    Chamberlain, J.S.4
  • 43
    • 0026638903 scopus 로고
    • The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy
    • Danko I., Chapman V., and Wolff J.A. The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy. Pediatr. Res. 32 (1992) 128-131
    • (1992) Pediatr. Res. , vol.32 , pp. 128-131
    • Danko, I.1    Chapman, V.2    Wolff, J.A.3
  • 45
    • 33947258069 scopus 로고    scopus 로고
    • Pathophysiology of duchenne muscular dystrophy: Current hypotheses
    • Deconinck N., and Dan B. Pathophysiology of duchenne muscular dystrophy: Current hypotheses. Pediatr. Neurol. 36 (2007) 1-7
    • (2007) Pediatr. Neurol. , vol.36 , pp. 1-7
    • Deconinck, N.1    Dan, B.2
  • 49
    • 0035726738 scopus 로고    scopus 로고
    • Tibialis anterior muscles in mdx mice are highly susceptible to contraction-induced injury
    • Dellorusso C., Crawford R.W., Chamberlain J.S., and Brooks S.V. Tibialis anterior muscles in mdx mice are highly susceptible to contraction-induced injury. J. Muscle Res. Cell Motil. 22 (2001) 467-475
    • (2001) J. Muscle Res. Cell Motil. , vol.22 , pp. 467-475
    • Dellorusso, C.1    Crawford, R.W.2    Chamberlain, J.S.3    Brooks, S.V.4
  • 52
    • 0025303118 scopus 로고
    • Dystrophin function
    • Emery A.E. Dystrophin function. Lancet 335 (1990) 1289
    • (1990) Lancet , vol.335 , pp. 1289
    • Emery, A.E.1
  • 54
    • 0038190993 scopus 로고    scopus 로고
    • Costameres: The Achilles' heel of Herculean muscle
    • Ervasti J.M. Costameres: The Achilles' heel of Herculean muscle. J. Biol. Chem. 278 (2003) 13591-13594
    • (2003) J. Biol. Chem. , vol.278 , pp. 13591-13594
    • Ervasti, J.M.1
  • 55
    • 33846271135 scopus 로고    scopus 로고
    • Dystrophin, its interactions with other proteins, and implications for muscular dystrophy
    • Ervasti J.M. Dystrophin, its interactions with other proteins, and implications for muscular dystrophy. Biochim. Biophys. Acta. 1772 (2007) 108-117
    • (2007) Biochim. Biophys. Acta. , vol.1772 , pp. 108-117
    • Ervasti, J.M.1
  • 56
    • 0025272250 scopus 로고
    • Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle
    • Ervasti J.M., Ohlendieck K., Kahl S.D., Gaver M.G., and Campbell K.P. Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle. Nature 345 (1990) 315-319
    • (1990) Nature , vol.345 , pp. 315-319
    • Ervasti, J.M.1    Ohlendieck, K.2    Kahl, S.D.3    Gaver, M.G.4    Campbell, K.P.5
  • 61
    • 33749071459 scopus 로고    scopus 로고
    • The role of Ca2+ in muscle cell damage
    • Gissel H. The role of Ca2+ in muscle cell damage. Ann. N. Y. Acad. Sci. 1066 (2005) 166-180
    • (2005) Ann. N. Y. Acad. Sci. , vol.1066 , pp. 166-180
    • Gissel, H.1
  • 62
    • 0026937998 scopus 로고
    • Expression of four alternative dystrophin transcripts in brain regions regulated by different promoters
    • Gorecki D.C., Monaco A.P., Derry J.M., Walker A.P., Barnard E.A., and Barnard P.J. Expression of four alternative dystrophin transcripts in brain regions regulated by different promoters. Hum. Mol. Genet. 1 (1992) 505-510
    • (1992) Hum. Mol. Genet. , vol.1 , pp. 505-510
    • Gorecki, D.C.1    Monaco, A.P.2    Derry, J.M.3    Walker, A.P.4    Barnard, E.A.5    Barnard, P.J.6
  • 64
    • 0030848338 scopus 로고    scopus 로고
    • Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: A model for Duchenne muscular dystrophy
    • Grady R.M., Teng H., Nichol M.C., Cunningham J.C., Wilkinson R.S., and Sanes J.R. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: A model for Duchenne muscular dystrophy. Cell 90 (1997) 729-738
    • (1997) Cell , vol.90 , pp. 729-738
    • Grady, R.M.1    Teng, H.2    Nichol, M.C.3    Cunningham, J.C.4    Wilkinson, R.S.5    Sanes, J.R.6
  • 67
    • 33747383933 scopus 로고    scopus 로고
    • Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue
    • Haenggi T., and Fritschy J.M. Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue. Cell Mol. Life Sci. 63 (2006) 1614-1631
    • (2006) Cell Mol. Life Sci. , vol.63 , pp. 1614-1631
    • Haenggi, T.1    Fritschy, J.M.2
  • 68
    • 34147109662 scopus 로고    scopus 로고
    • PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy
    • Handschin C., Kobayashi Y.M., Chin S., Seale P., Campbell K.P., and Spiegelman B.M. PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Genes Dev. 21 (2007) 770-783
    • (2007) Genes Dev. , vol.21 , pp. 770-783
    • Handschin, C.1    Kobayashi, Y.M.2    Chin, S.3    Seale, P.4    Campbell, K.P.5    Spiegelman, B.M.6
  • 69
    • 14644423890 scopus 로고    scopus 로고
    • NO/redox disequilibrium in the failing heart and cardiovascular system
    • Hare J.M., and Stamler J.S. NO/redox disequilibrium in the failing heart and cardiovascular system. J. Clin. Invest. 115 (2005) 509-517
    • (2005) J. Clin. Invest. , vol.115 , pp. 509-517
    • Hare, J.M.1    Stamler, J.S.2
  • 70
    • 0026689754 scopus 로고
    • [Intramuscular degeneration process in Duchenne muscular dystrophy-investigation by longitudinal MR imaging of the skeletal muscles]
    • Hasegawa T., Matsumura K., Hashimoto T., Ikehira H., Fukuda H., and Tateno Y. [Intramuscular degeneration process in Duchenne muscular dystrophy-investigation by longitudinal MR imaging of the skeletal muscles]. Rinsho Shinkeigaku 32 (1992) 333-335
    • (1992) Rinsho Shinkeigaku , vol.32 , pp. 333-335
    • Hasegawa, T.1    Matsumura, K.2    Hashimoto, T.3    Ikehira, H.4    Fukuda, H.5    Tateno, Y.6
  • 71
    • 33645355101 scopus 로고    scopus 로고
    • Utrophin upregulation in Duchenne muscular dystrophy
    • Hirst R.C., McCullagh K.J., and Davies K.E. Utrophin upregulation in Duchenne muscular dystrophy. Acta. Myol. 24 (2005) 209-216
    • (2005) Acta. Myol. , vol.24 , pp. 209-216
    • Hirst, R.C.1    McCullagh, K.J.2    Davies, K.E.3
  • 72
    • 0023614188 scopus 로고
    • Dystrophin: The protein product of the Duchenne muscular dystrophy locus
    • Hoffman E.P., Brown Jr. R.H., and Kunkel L.M. Dystrophin: The protein product of the Duchenne muscular dystrophy locus. Cell 51 (1987) 919-928
    • (1987) Cell , vol.51 , pp. 919-928
    • Hoffman, E.P.1    Brown Jr., R.H.2    Kunkel, L.M.3
  • 74
    • 0029820310 scopus 로고    scopus 로고
    • Differential expression of dystrophin isoforms in strains of mdx mice with different mutations
    • Im W.B., Phelps S.F., Copen E.H., Adams E.G., Slightom J.L., and Chamberlain J.S. Differential expression of dystrophin isoforms in strains of mdx mice with different mutations. Hum. Mol. Genet. 5 (1996) 1149-1153
    • (1996) Hum. Mol. Genet. , vol.5 , pp. 1149-1153
    • Im, W.B.1    Phelps, S.F.2    Copen, E.H.3    Adams, E.G.4    Slightom, J.L.5    Chamberlain, J.S.6
  • 76
    • 33645359475 scopus 로고    scopus 로고
    • Gene therapy for Duchenne muscular dystrophy: AAV leads the way
    • Judge L.M., and Chamberlain J.S. Gene therapy for Duchenne muscular dystrophy: AAV leads the way. Acta. Myol. 24 (2005) 184-193
    • (2005) Acta. Myol. , vol.24 , pp. 184-193
    • Judge, L.M.1    Chamberlain, J.S.2
  • 78
    • 0038725634 scopus 로고    scopus 로고
    • Pharmacological strategies for muscular dystrophy
    • Khurana T.S., and Davies K.E. Pharmacological strategies for muscular dystrophy. Nat. Rev. Drug Discov. 2 (2003) 379-390
    • (2003) Nat. Rev. Drug Discov. , vol.2 , pp. 379-390
    • Khurana, T.S.1    Davies, K.E.2
  • 79
    • 33745183090 scopus 로고    scopus 로고
    • Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophy
    • Kleopa K.A., Drousiotou A., Mavrikiou E., Ormiston A., and Kyriakides T. Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophy. Hum. Mol. Genet. 15 (2006) 1623-1628
    • (2006) Hum. Mol. Genet. , vol.15 , pp. 1623-1628
    • Kleopa, K.A.1    Drousiotou, A.2    Mavrikiou, E.3    Ormiston, A.4    Kyriakides, T.5
  • 80
    • 0025217703 scopus 로고
    • Detailed analysis of the repeat domain of dystrophin reveals four potential hinge segments that may confer flexibility
    • Koenig M., and Kunkel L.M. Detailed analysis of the repeat domain of dystrophin reveals four potential hinge segments that may confer flexibility. J. Biol. Chem. 265 (1990) 4560-4566
    • (1990) J. Biol. Chem. , vol.265 , pp. 4560-4566
    • Koenig, M.1    Kunkel, L.M.2
  • 83
    • 34249108083 scopus 로고    scopus 로고
    • Asymmetric self-renewal and commitment of satellite stem cells in muscle
    • Kuang S., Kuroda K., Le Grand F., and Rudnicki M.A. Asymmetric self-renewal and commitment of satellite stem cells in muscle. Cell 129 (2007) 999-1010
    • (2007) Cell , vol.129 , pp. 999-1010
    • Kuang, S.1    Kuroda, K.2    Le Grand, F.3    Rudnicki, M.A.4
  • 84
    • 0027717714 scopus 로고
    • Dystrophin deficiency is associated with myotendinous junction defects in prenecrotic and fully regenerated skeletal muscle
    • Law D.J., and Tidball J.G. Dystrophin deficiency is associated with myotendinous junction defects in prenecrotic and fully regenerated skeletal muscle. Am. J. Pathol. 142 (1993) 1513-1523
    • (1993) Am. J. Pathol. , vol.142 , pp. 1513-1523
    • Law, D.J.1    Tidball, J.G.2
  • 85
    • 0028264218 scopus 로고
    • Talin, vinculin and DRP (utrophin) concentrations are increased at mdx myotendinous junctions following onset of necrosis
    • Law D.J., Allen D.L., and Tidball J.G. Talin, vinculin and DRP (utrophin) concentrations are increased at mdx myotendinous junctions following onset of necrosis. J. Cell Sci. 107 Pt 6 (1994) 1477-1483
    • (1994) J. Cell Sci. , vol.107 , Issue.PART 6 , pp. 1477-1483
    • Law, D.J.1    Allen, D.L.2    Tidball, J.G.3
  • 86
    • 0027366990 scopus 로고
    • Monoclonal antibodies against the muscle-specific N-terminus of dystrophin: Characterization of dystrophin in a muscular dystrophy patient with a frameshift deletion of exons 3-7
    • Le T.T., Nguyen T.M., Love D.R., Helliwell T.R., Davies K.E., and Morris G.E. Monoclonal antibodies against the muscle-specific N-terminus of dystrophin: Characterization of dystrophin in a muscular dystrophy patient with a frameshift deletion of exons 3-7. Am. J. Hum. Genet. 53 (1993) 131-139
    • (1993) Am. J. Hum. Genet. , vol.53 , pp. 131-139
    • Le, T.T.1    Nguyen, T.M.2    Love, D.R.3    Helliwell, T.R.4    Davies, K.E.5    Morris, G.E.6
  • 88
    • 36749061251 scopus 로고    scopus 로고
    • Skeletal muscle satellite cells and adult myogenesis
    • Le Grand F., and Rudnicki M.A. Skeletal muscle satellite cells and adult myogenesis. Curr. Opin. Cell Biol. 19 (2007) 628-633
    • (2007) Curr. Opin. Cell Biol. , vol.19 , pp. 628-633
    • Le Grand, F.1    Rudnicki, M.A.2
  • 89
    • 0037458618 scopus 로고    scopus 로고
    • Interaction of dystrophin rod domain with membrane phospholipids
    • Evidence of a close proximity between tryptophan residues and lipids
    • Le Rumeur E., Fichou Y., Pottier S., Gaboriau F., Rondeau-Mouro C., Vincent M., Gallay J., and Bondon A. Interaction of dystrophin rod domain with membrane phospholipids. Evidence of a close proximity between tryptophan residues and lipids. J. Biol. Chem. 278 (2003) 5993-6001
    • (2003) J. Biol. Chem. , vol.278 , pp. 5993-6001
    • Le Rumeur, E.1    Fichou, Y.2    Pottier, S.3    Gaboriau, F.4    Rondeau-Mouro, C.5    Vincent, M.6    Gallay, J.7    Bondon, A.8
  • 90
    • 0028937525 scopus 로고
    • Dp140: A novel 140 kDa CNS transcript from the dystrophin locus
    • Lidov H.G., Selig S., and Kunkel L.M. Dp140: A novel 140 kDa CNS transcript from the dystrophin locus. Hum. Mol. Genet. 4 (1995) 329-335
    • (1995) Hum. Mol. Genet. , vol.4 , pp. 329-335
    • Lidov, H.G.1    Selig, S.2    Kunkel, L.M.3
  • 93
    • 4143054600 scopus 로고    scopus 로고
    • Role of contraction-induced injury in the mechanisms of muscle damage in muscular dystrophy
    • Lynch G.S. Role of contraction-induced injury in the mechanisms of muscle damage in muscular dystrophy. Clin. Exp. Pharmacol. Physiol. 31 (2004) 557-561
    • (2004) Clin. Exp. Pharmacol. Physiol. , vol.31 , pp. 557-561
    • Lynch, G.S.1
  • 94
    • 0026323638 scopus 로고
    • Structure and function of the neuromuscular junction in young adult mdx mice
    • Lyons P.R., and Slater C.R. Structure and function of the neuromuscular junction in young adult mdx mice. J. Neurocytol. 20 (1991) 969-981
    • (1991) J. Neurocytol. , vol.20 , pp. 969-981
    • Lyons, P.R.1    Slater, C.R.2
  • 95
    • 0028817970 scopus 로고
    • Visualization of dystrophic muscle fibers in mdx mouse by vital staining with Evans blue: Evidence of apoptosis in dystrophin-deficient muscle
    • Matsuda R., Nishikawa A., and Tanaka H. Visualization of dystrophic muscle fibers in mdx mouse by vital staining with Evans blue: Evidence of apoptosis in dystrophin-deficient muscle. J. Biochem. (Tokyo) 118 (1995) 959-964
    • (1995) J. Biochem. (Tokyo) , vol.118 , pp. 959-964
    • Matsuda, R.1    Nishikawa, A.2    Tanaka, H.3
  • 96
    • 0026621608 scopus 로고
    • Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
    • Matsumura K., Ervasti J.M., Ohlendieck K., Kahl S.D., and Campbell K.P. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature 360 (1992) 588-591
    • (1992) Nature , vol.360 , pp. 588-591
    • Matsumura, K.1    Ervasti, J.M.2    Ohlendieck, K.3    Kahl, S.D.4    Campbell, K.P.5
  • 97
    • 0028044626 scopus 로고
    • Immunohistochemical analysis of dystrophin-associated proteins in Becker/Duchenne muscular dystrophy with huge in-frame deletions in the NH2-terminal and rod domains of dystrophin
    • Matsumura K., Burghes A.H., Mora M., Tome F.M., Morandi L., Cornello F., Leturcq F., Jeanpierre M., Kaplan J.C., Reinert P., et al. Immunohistochemical analysis of dystrophin-associated proteins in Becker/Duchenne muscular dystrophy with huge in-frame deletions in the NH2-terminal and rod domains of dystrophin. J. Clin. Invest. 93 (1994) 99-105
    • (1994) J. Clin. Invest. , vol.93 , pp. 99-105
    • Matsumura, K.1    Burghes, A.H.2    Mora, M.3    Tome, F.M.4    Morandi, L.5    Cornello, F.6    Leturcq, F.7    Jeanpierre, M.8    Kaplan, J.C.9    Reinert, P.10
  • 98
    • 0027385135 scopus 로고
    • Age-related changes in replication of myogenic cells in mdx mice: Quantitative autoradiographic studies
    • McGeachie J.K., Grounds M.D., Partridge T.A., and Morgan J.E. Age-related changes in replication of myogenic cells in mdx mice: Quantitative autoradiographic studies. J. Neurol. Sci. 119 (1993) 169-179
    • (1993) J. Neurol. Sci. , vol.119 , pp. 169-179
    • McGeachie, J.K.1    Grounds, M.D.2    Partridge, T.A.3    Morgan, J.E.4
  • 100
    • 0028092013 scopus 로고
    • Molecular diagnosis and modern management of Duchenne muscular dystrophy
    • Miller R.G., and Hoffman E.P. Molecular diagnosis and modern management of Duchenne muscular dystrophy. Neurol. Clin. 12 (1994) 699-725
    • (1994) Neurol. Clin. , vol.12 , pp. 699-725
    • Miller, R.G.1    Hoffman, E.P.2
  • 101
    • 0027248618 scopus 로고
    • Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch
    • Moens P., Baatsen P.H., and Marechal G. Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch. J. Muscle Res. Cell Motil. 14 (1993) 446-451
    • (1993) J. Muscle Res. Cell Motil. , vol.14 , pp. 446-451
    • Moens, P.1    Baatsen, P.H.2    Marechal, G.3
  • 102
    • 0023718118 scopus 로고
    • An explanation for the phenotypic differences between patients bearing partial deletions of the DMD locus
    • Monaco A.P., Bertelson C.J., Liechti-Gallati S., Moser H., and Kunkel L.M. An explanation for the phenotypic differences between patients bearing partial deletions of the DMD locus. Genomics 2 (1988) 90-95
    • (1988) Genomics , vol.2 , pp. 90-95
    • Monaco, A.P.1    Bertelson, C.J.2    Liechti-Gallati, S.3    Moser, H.4    Kunkel, L.M.5
  • 104
    • 0027466562 scopus 로고
    • Long-term persistence and migration of myogenic cells injected into pre-irradiated muscles of mdx mice
    • Morgan J.E., Pagel C.N., Sherratt T., and Partridge T.A. Long-term persistence and migration of myogenic cells injected into pre-irradiated muscles of mdx mice. J. Neurol. Sci. 115 (1993) 191-200
    • (1993) J. Neurol. Sci. , vol.115 , pp. 191-200
    • Morgan, J.E.1    Pagel, C.N.2    Sherratt, T.3    Partridge, T.A.4
  • 105
    • 34648854432 scopus 로고    scopus 로고
    • Genetic treatments in muscular dystrophies
    • Muntoni F., and Wells D. Genetic treatments in muscular dystrophies. Curr. Opin. Neurol. 20 (2007) 590-594
    • (2007) Curr. Opin. Neurol. , vol.20 , pp. 590-594
    • Muntoni, F.1    Wells, D.2
  • 107
    • 0344420060 scopus 로고    scopus 로고
    • Dystrophin and mutations: One gene, several proteins, multiple phenotypes
    • Muntoni F., Torelli S., and Ferlini A. Dystrophin and mutations: One gene, several proteins, multiple phenotypes. Lancet. Neurol. 2 (2003) 731-740
    • (2003) Lancet. Neurol. , vol.2 , pp. 731-740
    • Muntoni, F.1    Torelli, S.2    Ferlini, A.3
  • 108
    • 0026073326 scopus 로고
    • [Magnetic resonance imaging of skeletal muscle in patients with Duchenne muscular dystrophy-serial axial and sagittal section studies]
    • Nagao H., Morimoto T., Sano N., Takahashi M., Nagai H., Tawa R., Yoshimatsu M., Woo Y.J., and Matsuda H. [Magnetic resonance imaging of skeletal muscle in patients with Duchenne muscular dystrophy-serial axial and sagittal section studies]. No To Hattatsu 23 (1991) 39-43
    • (1991) No To Hattatsu , vol.23 , pp. 39-43
    • Nagao, H.1    Morimoto, T.2    Sano, N.3    Takahashi, M.4    Nagai, H.5    Tawa, R.6    Yoshimatsu, M.7    Woo, Y.J.8    Matsuda, H.9
  • 109
    • 0037440706 scopus 로고    scopus 로고
    • Interactions between neutrophils and macrophages promote macrophage killing of rat muscle cells in vitro
    • Nguyen H.X., and Tidball J.G. Interactions between neutrophils and macrophages promote macrophage killing of rat muscle cells in vitro. J. Physiol. 547 (2003) 125-132
    • (2003) J. Physiol. , vol.547 , pp. 125-132
    • Nguyen, H.X.1    Tidball, J.G.2
  • 110
    • 0036485777 scopus 로고    scopus 로고
    • Muscle lesions associated with dystrophin deficiency in neonatal golden retriever puppies
    • Nguyen F., Cherel Y., Guigand L., Goubault-Leroux I., and Wyers M. Muscle lesions associated with dystrophin deficiency in neonatal golden retriever puppies. J. Comp. Pathol. 126 (2002) 100-108
    • (2002) J. Comp. Pathol. , vol.126 , pp. 100-108
    • Nguyen, F.1    Cherel, Y.2    Guigand, L.3    Goubault-Leroux, I.4    Wyers, M.5
  • 113
    • 0032969798 scopus 로고    scopus 로고
    • Creatine kinase, cell membrane and Duchenne muscular dystrophy
    • Ozawa E., Hagiwara Y., and Yoshida M. Creatine kinase, cell membrane and Duchenne muscular dystrophy. Mol. Cell Biochem. 190 (1999) 143-151
    • (1999) Mol. Cell Biochem. , vol.190 , pp. 143-151
    • Ozawa, E.1    Hagiwara, Y.2    Yoshida, M.3
  • 114
    • 45749126999 scopus 로고    scopus 로고
    • Hematopoietic cell transplantation provides an immune-tolerant platform for myoblast transplantation in dystrophic dogs
    • Parker M.H., Kuhr C., Tapscott S.J., and Storb R. Hematopoietic cell transplantation provides an immune-tolerant platform for myoblast transplantation in dystrophic dogs. Mol. Ther. 16 (2008) 1340-1346
    • (2008) Mol. Ther. , vol.16 , pp. 1340-1346
    • Parker, M.H.1    Kuhr, C.2    Tapscott, S.J.3    Storb, R.4
  • 115
    • 0003020112 scopus 로고    scopus 로고
    • Models of dystrophinopathy, pathological mechanisms and assessment of therapies
    • Brown S.C., and Lucy J.A. (Eds), Cambridge University Press, Cambridge
    • Partridge T.A. Models of dystrophinopathy, pathological mechanisms and assessment of therapies. In: Brown S.C., and Lucy J.A. (Eds). Dystrophin: Gene Protein and Cell Biology (1997), Cambridge University Press, Cambridge 310-311
    • (1997) Dystrophin: Gene Protein and Cell Biology , pp. 310-311
    • Partridge, T.A.1
  • 116
    • 33646083660 scopus 로고    scopus 로고
    • Disciplining the stem cell into myogenesis
    • Partridge T. Disciplining the stem cell into myogenesis. N. Engl. J. Med. 354 (2006) 1844-1845
    • (2006) N. Engl. J. Med. , vol.354 , pp. 1844-1845
    • Partridge, T.1
  • 120
    • 0032007205 scopus 로고    scopus 로고
    • Muscle cells from mdx mice have an increased susceptibility to oxidative stress
    • Rando T.A., Disatnik M.H., Yu Y., and Franco A. Muscle cells from mdx mice have an increased susceptibility to oxidative stress. Neuromuscul. Disord. 8 (1998) 14-21
    • (1998) Neuromuscul. Disord. , vol.8 , pp. 14-21
    • Rando, T.A.1    Disatnik, M.H.2    Yu, Y.3    Franco, A.4
  • 121
    • 0032915455 scopus 로고    scopus 로고
    • The WW domain of dystrophin requires EF-hands region to interact with beta-dystroglycan
    • Rentschler S., Linn H., Deininger K., Bedford M.T., Espanel X., and Sudol M. The WW domain of dystrophin requires EF-hands region to interact with beta-dystroglycan. J. Biol. Chem. 380 (1999) 431-442
    • (1999) J. Biol. Chem. , vol.380 , pp. 431-442
    • Rentschler, S.1    Linn, H.2    Deininger, K.3    Bedford, M.T.4    Espanel, X.5    Sudol, M.6
  • 123
    • 36448994441 scopus 로고    scopus 로고
    • A translational approach for limb vascular delivery of the micro-dystrophin gene without high volume or high pressure for treatment of Duchenne muscular dystrophy
    • Rodino-Klapac L.R., Janssen P.M., Montgomery C.L., Coley B.D., Chicoine L.G., Clark K.R., and Mendell J.R. A translational approach for limb vascular delivery of the micro-dystrophin gene without high volume or high pressure for treatment of Duchenne muscular dystrophy. J. Transl. Med. 5 (2007) 45
    • (2007) J. Transl. Med. , vol.5 , pp. 45
    • Rodino-Klapac, L.R.1    Janssen, P.M.2    Montgomery, C.L.3    Coley, B.D.4    Chicoine, L.G.5    Clark, K.R.6    Mendell, J.R.7
  • 124
    • 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. Utrophin binds laterally along actin filaments and can couple costameric actin with sarcolemma when overexpressed in dystrophin-deficient muscle. Mol. Biol. Cell 13 (2002) 1512-1521
    • (2002) Mol. Biol. Cell , vol.13 , pp. 1512-1521
    • Rybakova, I.N.1    Patel, J.R.2    Davies, K.E.3    Yurchenco, P.D.4    Ervasti, J.M.5
  • 125
    • 33744507184 scopus 로고    scopus 로고
    • Dystrophin and Utrophin Bind Actin through Distinct Modes of Contact
    • Rybakova I.N., Humston J.L., Sonnemann K.J., and Ervasti J.M. Dystrophin and Utrophin Bind Actin through Distinct Modes of Contact. J. Biol. Chem. 281 (2006) 9996-10001
    • (2006) J. Biol. Chem. , vol.281 , pp. 9996-10001
    • Rybakova, I.N.1    Humston, J.L.2    Sonnemann, K.J.3    Ervasti, J.M.4
  • 126
    • 33646524933 scopus 로고    scopus 로고
    • Structural cooperativity in spectrin type repeats motifs of dystrophin
    • Saadat L., Pittman L., and Menhart N. Structural cooperativity in spectrin type repeats motifs of dystrophin. Biochim. Biophys. Acta. 1764 (2006) 943-954
    • (2006) Biochim. Biophys. Acta. , vol.1764 , pp. 943-954
    • Saadat, L.1    Pittman, L.2    Menhart, N.3
  • 134
    • 0020306184 scopus 로고
    • Skeletal muscle satellite cells: Changes in proliferation potential as a function of age
    • Schultz E., and Lipton B.H. Skeletal muscle satellite cells: Changes in proliferation potential as a function of age. Mech. Ageing Dev. 20 (1982) 377-383
    • (1982) Mech. Ageing Dev. , vol.20 , pp. 377-383
    • Schultz, E.1    Lipton, B.H.2
  • 139
    • 3042624687 scopus 로고    scopus 로고
    • Structural determinants of the reliability of synaptic transmission at the vertebrate neuromuscular junction
    • Slater C.R. Structural determinants of the reliability of synaptic transmission at the vertebrate neuromuscular junction. J. Neurocytol. 32 (2003) 505-522
    • (2003) J. Neurocytol. , vol.32 , pp. 505-522
    • Slater, C.R.1
  • 140
    • 0036798005 scopus 로고    scopus 로고
    • Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology
    • Spencer M.J., and Mellgren R.L. Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology. Hum. Mol. Genet. 11 (2002) 2645-2655
    • (2002) Hum. Mol. Genet. , vol.11 , pp. 2645-2655
    • Spencer, M.J.1    Mellgren, R.L.2
  • 141
    • 34248172959 scopus 로고    scopus 로고
    • Mechanosensitive channel properties and membrane mechanics in mouse dystrophic myotubes
    • Suchyna T.M., and Sachs F. Mechanosensitive channel properties and membrane mechanics in mouse dystrophic myotubes. J. Physiol. 581 (2007) 369-387
    • (2007) J. Physiol. , vol.581 , pp. 369-387
    • Suchyna, T.M.1    Sachs, F.2
  • 143
    • 0028101457 scopus 로고
    • Amino-terminal deletion of 53% of dystrophin results in an intermediate Duchenne-Becker muscular dystrophy phenotype
    • Takeshima Y., Nishio H., Narita N., Wada H., Ishikawa Y., Ishikawa Y., Minami R., Nakamura H., and Matsuo M. Amino-terminal deletion of 53% of dystrophin results in an intermediate Duchenne-Becker muscular dystrophy phenotype. Neurology 44 (1994) 1648-1651
    • (1994) Neurology , vol.44 , pp. 1648-1651
    • Takeshima, Y.1    Nishio, H.2    Narita, N.3    Wada, H.4    Ishikawa, Y.5    Ishikawa, Y.6    Minami, R.7    Nakamura, H.8    Matsuo, M.9
  • 144
    • 34147152905 scopus 로고    scopus 로고
    • The role of free radicals in the pathophysiology of muscular dystrophy
    • Tidball J.G., and Wehling-Henricks M. The role of free radicals in the pathophysiology of muscular dystrophy. J. Appl. Physiol. 102 (2007) 1677-1686
    • (2007) J. Appl. Physiol. , vol.102 , pp. 1677-1686
    • Tidball, J.G.1    Wehling-Henricks, M.2
  • 145
    • 0029906168 scopus 로고    scopus 로고
    • 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., and Davies K.E. Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene. Nature 384 (1996) 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
  • 147
    • 0026066793 scopus 로고
    • Canine X-linked muscular dystrophy: Selective involvement of muscles in neonatal dogs
    • Valentine B.A., and Cooper B.J. Canine X-linked muscular dystrophy: Selective involvement of muscles in neonatal dogs. Neuromuscul. Disord. 1 (1991) 31-38
    • (1991) Neuromuscul. Disord. , vol.1 , pp. 31-38
    • Valentine, B.A.1    Cooper, B.J.2
  • 148
    • 0024263367 scopus 로고
    • Canine X-linked muscular dystrophy. An animal model of Duchenne muscular dystrophy: Clinical studies
    • Valentine B.A., Cooper B.J., de Lahunta A., O'Quinn R., and Blue J.T. Canine X-linked muscular dystrophy. An animal model of Duchenne muscular dystrophy: Clinical studies. J. Neurol. Sci. 88 (1988) 69-81
    • (1988) J. Neurol. Sci. , vol.88 , pp. 69-81
    • Valentine, B.A.1    Cooper, B.J.2    de Lahunta, A.3    O'Quinn, R.4    Blue, J.T.5
  • 150
    • 0037119993 scopus 로고    scopus 로고
    • Involvement of TRPC in the abnormal calcium influx observed in dystrophic (mdx) mouse skeletal muscle fibers
    • Vandebrouck C., Martin D., Colson-Van Schoor M., Debaix H., and Gailly P. Involvement of TRPC in the abnormal calcium influx observed in dystrophic (mdx) mouse skeletal muscle fibers. J. Cell Biol. 158 (2002) 1089-1096
    • (2002) J. Cell Biol. , vol.158 , pp. 1089-1096
    • Vandebrouck, C.1    Martin, D.2    Colson-Van Schoor, M.3    Debaix, H.4    Gailly, P.5
  • 151
    • 0033994291 scopus 로고    scopus 로고
    • Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene
    • Wakefield P.M., Tinsley J.M., Wood M.J., Gilbert R., Karpati G., and Davies K.E. Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene. Gene. Ther. 7 (2000) 201-204
    • (2000) Gene. Ther. , vol.7 , pp. 201-204
    • Wakefield, P.M.1    Tinsley, J.M.2    Wood, M.J.3    Gilbert, R.4    Karpati, G.5    Davies, K.E.6
  • 153
    • 34249276065 scopus 로고    scopus 로고
    • Sustained AAV-mediated dystrophin expression in a canine model of Duchenne muscular dystrophy with a brief course of immunosuppression
    • Wang Z., Kuhr C.S., Allen J.M., Blankinship M., Gregorevic P., Chamberlain J.S., Tapscott S.J., and Storb R. Sustained AAV-mediated dystrophin expression in a canine model of Duchenne muscular dystrophy with a brief course of immunosuppression. Mol. Ther. 15 (2007) 1160-1166
    • (2007) Mol. Ther. , vol.15 , pp. 1160-1166
    • Wang, Z.1    Kuhr, C.S.2    Allen, J.M.3    Blankinship, M.4    Gregorevic, P.5    Chamberlain, J.S.6    Tapscott, S.J.7    Storb, R.8
  • 154
    • 0036566558 scopus 로고    scopus 로고
    • Expression of Dp260 in muscle tethers the actin cytoskeleton to the dystrophin-glycoprotein complex and partially prevents dystrophy
    • Warner L.E., DelloRusso C., Crawford R.W., Rybakova I.N., Patel J.R., Ervasti J.M., and Chamberlain J.S. Expression of Dp260 in muscle tethers the actin cytoskeleton to the dystrophin-glycoprotein complex and partially prevents dystrophy. Hum. Mol. Genet. 11 (2002) 1095-1105
    • (2002) Hum. Mol. Genet. , vol.11 , pp. 1095-1105
    • Warner, L.E.1    DelloRusso, C.2    Crawford, R.W.3    Rybakova, I.N.4    Patel, J.R.5    Ervasti, J.M.6    Chamberlain, J.S.7
  • 155
    • 0025646136 scopus 로고
    • Accelerated age-related decline in replicative life-span of Duchenne muscular dystrophy myoblasts: Implications for cell and gene therapy
    • Webster C., and Blau H.M. Accelerated age-related decline in replicative life-span of Duchenne muscular dystrophy myoblasts: Implications for cell and gene therapy. Somat. Cell Mol. Genet. 16 (1990) 557-565
    • (1990) Somat. Cell Mol. Genet. , vol.16 , pp. 557-565
    • Webster, C.1    Blau, H.M.2
  • 156
    • 0035494438 scopus 로고    scopus 로고
    • A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice
    • Wehling M., Spencer M.J., and Tidball J.G. A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice. J. Cell Biol. 155 (2001) 123-131
    • (2001) J. Cell Biol. , vol.155 , pp. 123-131
    • Wehling, M.1    Spencer, M.J.2    Tidball, J.G.3
  • 157
    • 0037160098 scopus 로고    scopus 로고
    • A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle
    • Weir A.P., Burton E.A., Harrod G., and Davies K.E. A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle. J. Biol. Chem. 277 (2002) 45285-45290
    • (2002) J. Biol. Chem. , vol.277 , pp. 45285-45290
    • Weir, A.P.1    Burton, E.A.2    Harrod, G.3    Davies, K.E.4
  • 159
    • 45549084494 scopus 로고    scopus 로고
    • N-Acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice
    • Whitehead N.P., Pham C., Gervasio O.L., and Allen D.G. N-Acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice. J. Physiol. 586 (2008) 2003-2014
    • (2008) J. Physiol. , vol.586 , pp. 2003-2014
    • Whitehead, N.P.1    Pham, C.2    Gervasio, O.L.3    Allen, D.G.4
  • 160
    • 0028833771 scopus 로고
    • Frameshift deletions of exons 3-7 and revertant fibers in Duchenne muscular dystrophy: Mechanisms of dystrophin production
    • Winnard A.V., Mendell J.R., Prior T.W., Florence J., and Burghes A.H. Frameshift deletions of exons 3-7 and revertant fibers in Duchenne muscular dystrophy: Mechanisms of dystrophin production. Am. J. Hum. Genet. 56 (1995) 158-166
    • (1995) Am. J. Hum. Genet. , vol.56 , pp. 158-166
    • Winnard, A.V.1    Mendell, J.R.2    Prior, T.W.3    Florence, J.4    Burghes, A.H.5
  • 161
    • 12744268414 scopus 로고    scopus 로고
    • Effects of stretch-activated channel blockers on [Ca2+]i and muscle damage in the mdx mouse
    • Yeung E.W., Whitehead N.P., Suchyna T.M., Gottlieb P.A., Sachs F., and Allen D.G. Effects of stretch-activated channel blockers on [Ca2+]i and muscle damage in the mdx mouse. J. Physiol. 562 (2005) 367-380
    • (2005) J. Physiol. , vol.562 , pp. 367-380
    • Yeung, E.W.1    Whitehead, N.P.2    Suchyna, T.M.3    Gottlieb, P.A.4    Sachs, F.5    Allen, D.G.6
  • 162
    • 33745248418 scopus 로고    scopus 로고
    • C-terminal-truncated microdystrophin recruits dystrobrevin and syntrophin to the dystrophin-associated glycoprotein complex and reduces muscular dystrophy in symptomatic utrophin/dystrophin double-knockout mice
    • Yue Y., Liu M., and Duan D. C-terminal-truncated microdystrophin recruits dystrobrevin and syntrophin to the dystrophin-associated glycoprotein complex and reduces muscular dystrophy in symptomatic utrophin/dystrophin double-knockout mice. Mol. Ther. 14 (2006) 79-87
    • (2006) Mol. Ther. , vol.14 , pp. 79-87
    • Yue, Y.1    Liu, M.2    Duan, D.3
  • 163
    • 0026680201 scopus 로고
    • Dystrophin and a dystrophin-related protein in intrafusal muscle fibers, and neuromuscular and myotendinous junctions
    • Zhao J., Yoshioka K., Miyatake M., and Miike T. Dystrophin and a dystrophin-related protein in intrafusal muscle fibers, and neuromuscular and myotendinous junctions. Acta. Neuropathol. (Berl) 84 (1992) 141-146
    • (1992) Acta. Neuropathol. (Berl) , vol.84 , pp. 141-146
    • Zhao, J.1    Yoshioka, K.2    Miyatake, M.3    Miike, T.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.