메뉴 건너뛰기




Volumn 2010, Issue , 2010, Pages

Dystrophins, utrophins, and associated scaffolding complexes: Role in mammalian brain and implications for therapeutic strategies

Author keywords

[No Author keywords available]

Indexed keywords

DEFLAZACORT; DYSTROPHIN; DYSTROPHIN ASSOCIATED GLYCOPROTEIN COMPLEX; DYSTROPHIN ASSOCIATED PROTEIN COMPLEX; GENE PRODUCT; PREDNISOLONE; SOMATOMEDIN; UNCLASSIFIED DRUG; UTROPHIN;

EID: 77954545973     PISSN: 11107243     EISSN: 11107251     Source Type: Journal    
DOI: 10.1155/2010/849426     Document Type: Review
Times cited : (78)

References (205)
  • 1
    • 0023614188 scopus 로고
    • Dystrophin: The protein product of the Duchenne muscular dystrophy locus
    • Hoffman E. P., Brown R. H. Jr., Kunkel L. M., Dystrophin: the protein product of the Duchenne muscular dystrophy locus Cell 1987 51 6 919 928
    • (1987) Cell , vol.51 , Issue.6 , pp. 919-928
    • Hoffman, E.P.1    Brown Jr., R.H.2    Kunkel, L.M.3
  • 2
    • 0034643837 scopus 로고    scopus 로고
    • Poor verbal working memory across intellectual level in boys with Duchenne dystrophy
    • Hinton V. J., De Vivo B. C., Nereo N. E., Goldstein E., Stern Y., Poor verbal working memory across intellectual level in boys with Duchenne dystrophy Neurology 2000 54 11 2127 2132
    • (2000) Neurology , vol.54 , Issue.11 , pp. 2127-2132
    • Hinton, V.J.1    De Vivo, B.C.2    Nereo, N.E.3    Goldstein, E.4    Stern, Y.5
  • 3
    • 84887212371 scopus 로고    scopus 로고
    • Clinical heterogeneity of Duchenne muscular dystrophy (DMD): Definition of sub-phenotypes and predictive criteria by long-term follow-up
    • ARTICLE E4347
    • Desguerre I., Christov C., Mayer M., Clinical heterogeneity of Duchenne muscular dystrophy (DMD): definition of sub-phenotypes and predictive criteria by long-term follow-up PloS One 2009 4 2, article e4347
    • (2009) PloS One , vol.4 , pp. 2
    • Desguerre, I.1    Christov, C.2    Mayer, M.3
  • 4
    • 0036087342 scopus 로고    scopus 로고
    • Function and genetics of dystrophin and dystrophin-related proteins in muscle
    • Blake D. J., Weir A., Newey S. E., Davies K. E., Function and genetics of dystrophin and dystrophin-related proteins in muscle Physiological Reviews 2002 82 2 291 329
    • (2002) Physiological Reviews , vol.82 , Issue.2 , pp. 291-329
    • Blake, D.J.1    Weir, A.2    Newey, S.E.3    Davies, K.E.4
  • 5
    • 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., Fritschy J.-M., Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue Cellular and Molecular Life Sciences 2006 63 14 1614 1631
    • (2006) Cellular and Molecular Life Sciences , vol.63 , Issue.14 , pp. 1614-1631
    • Haenggi, T.1    Fritschy, J.-M.2
  • 7
    • 0027095821 scopus 로고
    • Cognitive functions in duchenne muscular dystrophy: A reappraisal and comparison with spinal muscular atrophy
    • Billard C., Gillet P., Signoret J. L., Cognitive functions in duchenne muscular dystrophy: a reappraisal and comparison with spinal muscular atrophy Neuromuscular Disorders 1992 2 5-6 371 378
    • (1992) Neuromuscular Disorders , vol.2 , Issue.56 , pp. 371-378
    • Billard, C.1    Gillet, P.2    Signoret, J.L.3
  • 8
    • 0034933730 scopus 로고    scopus 로고
    • Intelligence and Duchenne muscular dystrophy: Full-scale, verbal, and performance intelligence quotients
    • Cotton S., Voudouris N. J., Greenwood K. M., Intelligence and Duchenne muscular dystrophy: full-scale, verbal, and performance intelligence quotients Developmental Medicine and Child Neurology 2001 43 7 497 501
    • (2001) Developmental Medicine and Child Neurology , vol.43 , Issue.7 , pp. 497-501
    • Cotton, S.1    Voudouris, N.J.2    Greenwood, K.M.3
  • 10
    • 0142153391 scopus 로고    scopus 로고
    • Psychopathological assessment in children affected by Duchenne de Boulogne muscular dystrophy
    • Roccella M., Pace R., De Gregorio M. T., Psychopathological assessment in children affected by Duchenne de Boulogne muscular dystrophy Minerva Pediatrica 2003 55 3 267 276
    • (2003) Minerva Pediatrica , vol.55 , Issue.3 , pp. 267-276
    • Roccella, M.1    Pace, R.2    De Gregorio, M.T.3
  • 11
    • 0035109506 scopus 로고    scopus 로고
    • Selective deficits in verbal working memory associated with a known genetic etiology: The neuropsychological profile of Duchenne muscular dystrophy
    • Hinton V. J., De Vivo D. C., Nereo N. E., Goldstein E., Stern Y., Selective deficits in verbal working memory associated with a known genetic etiology: the neuropsychological profile of Duchenne muscular dystrophy Journal of the International Neuropsychological Society 2001 7 1 45 54
    • (2001) Journal of the International Neuropsychological Society , vol.7 , Issue.1 , pp. 45-54
    • Hinton, V.J.1    De Vivo, D.C.2    Nereo, N.E.3    Goldstein, E.4    Stern, Y.5
  • 12
    • 42449160246 scopus 로고    scopus 로고
    • Neuropsychiatric disorders in males with duchenne muscular dystrophy: Frequency rate of attention-deficit hyperactivity disorder (ADHD), autism spectrum disorder, and obsessive-compulsive disorder
    • Hendriksen J. G. M., Vles J. S. H., Neuropsychiatric disorders in males with duchenne muscular dystrophy: frequency rate of attention-deficit hyperactivity disorder (ADHD), autism spectrum disorder, and obsessive-compulsive disorder Journal of Child Neurology 2008 23 5 477 481
    • (2008) Journal of Child Neurology , vol.23 , Issue.5 , pp. 477-481
    • Hendriksen, J.G.M.1    Vles, J.S.H.2
  • 15
    • 67649716564 scopus 로고    scopus 로고
    • Neurobehavioral characteristics of children with duchenne muscular dystrophy
    • Donders J., Taneja C., Neurobehavioral characteristics of children with duchenne muscular dystrophy Child Neuropsychology 2009 15 3 295 304
    • (2009) Child Neuropsychology , vol.15 , Issue.3 , pp. 295-304
    • Donders, J.1    Taneja, C.2
  • 17
    • 0027190703 scopus 로고
    • The distribution of dystrophin in the murine central nervous system: An immunocytochemical study
    • Lidov H. G. W., Byers T. J., Kunkel L. M., The distribution of dystrophin in the murine central nervous system: an immunocytochemical study Neuroscience 1993 54 1 167 187
    • (1993) Neuroscience , vol.54 , Issue.1 , pp. 167-187
    • Lidov, H.G.W.1    Byers, T.J.2    Kunkel, L.M.3
  • 18
    • 0026338937 scopus 로고
    • Apparent association of mental retardation and specific patterns of deletions screened with probes cf56a and cf23a in Duchenne Muscular Dystrophy
    • Rapaport D., Passos-Bueno M. R., Brandao L., Love D., Vainzof M., Zatz M., Apparent association of mental retardation and specific patterns of deletions screened with probes cf56a and cf23a in Duchenne Muscular Dystrophy American Journal of Medical Genetics 1991 39 4 437 441
    • (1991) American Journal of Medical Genetics , vol.39 , Issue.4 , pp. 437-441
    • Rapaport, D.1    Passos-Bueno, M.R.2    Brandao, L.3    Love, D.4    Vainzof, M.5    Zatz, M.6
  • 19
    • 0027484533 scopus 로고
    • Point mutations at the carboxy terminus of the human dystrophin gene: Implications for an association with mental retardation in DMD patients
    • Lenk U., Hanke R., Thiele H., Speer A., Point mutations at the carboxy terminus of the human dystrophin gene: implications for an association with mental retardation in DMD patients Human Molecular Genetics 1993 2 11 1877 1881
    • (1993) Human Molecular Genetics , vol.2 , Issue.11 , pp. 1877-1881
    • Lenk, U.1    Hanke, R.2    Thiele, H.3    Speer, A.4
  • 21
    • 0033920086 scopus 로고    scopus 로고
    • Severe cognitive impairment in DMD: Obvious clinical indication for Dp71 isoform point mutation screening
    • Moizard M.-P., Toutain A., Fournier D., Severe cognitive impairment in DMD: obvious clinical indication for Dp71 isoform point mutation screening European Journal of Human Genetics 2000 8 7 552 556
    • (2000) European Journal of Human Genetics , vol.8 , Issue.7 , pp. 552-556
    • Moizard, M.-P.1    Toutain, A.2    Fournier, D.3
  • 22
    • 0033841262 scopus 로고    scopus 로고
    • Loss of Dp140 dystrophin isoform and intellectual impairment in Duchenne dystrophy
    • Felisari G., Boneschi F. M., Bardoni A., Loss of Dp140 dystrophin isoform and intellectual impairment in Duchenne dystrophy Neurology 2000 55 4 559 564
    • (2000) Neurology , vol.55 , Issue.4 , pp. 559-564
    • Felisari, G.1    Boneschi, F.M.2    Bardoni, A.3
  • 23
    • 70349573300 scopus 로고    scopus 로고
    • Analysis of Dp71 contribution in the severity of mental retardation through comparison of Duchenne and Becker patients differing by mutation consequences on Dp71 expression
    • Daoud F., Angeard N., Demerre B., Analysis of Dp71 contribution in the severity of mental retardation through comparison of Duchenne and Becker patients differing by mutation consequences on Dp71 expression Human Molecular Genetics 2009 18 20 3779 3794
    • (2009) Human Molecular Genetics , vol.18 , Issue.20 , pp. 3779-3794
    • Daoud, F.1    Angeard, N.2    Demerre, B.3
  • 24
    • 77649282617 scopus 로고    scopus 로고
    • Dystrophin gene mutation location and the risk of cognitive impairment in duchenne muscular dystrophy
    • ARTICLE E8803
    • Taylor P. J., Betts G. A., Maroulis S., Dystrophin gene mutation location and the risk of cognitive impairment in duchenne muscular dystrophy PloS One 2010 5 1, article e8803
    • (2010) PloS One , vol.5 , pp. 1
    • Taylor, P.J.1    Betts, G.A.2    Maroulis, S.3
  • 25
    • 0344420060 scopus 로고    scopus 로고
    • Dystrophin and mutations: One gene, several proteins, multiple phenotypes
    • Muntoni F., Torelli S., Ferlini A., Dystrophin and mutations: one gene, several proteins, multiple phenotypes Lancet Neurology 2003 2 12 731 740
    • (2003) Lancet Neurology , vol.2 , Issue.12 , pp. 731-740
    • Muntoni, F.1    Torelli, S.2    Ferlini, A.3
  • 26
    • 0027203989 scopus 로고
    • Integrated study of 100 patients with Xp21 linked muscular dystrophy using clinical, genetic, immunochemical, and histopathological data. Part 1. Trends across the clinical groups
    • Nicholson L. V. B., Johnson M. A., Bushby K. M. D., Integrated study of 100 patients with Xp21 linked muscular dystrophy using clinical, genetic, immunochemical, and histopathological data. Part 1. Trends across the clinical groups Journal of Medical Genetics 1993 30 9 728 736
    • (1993) Journal of Medical Genetics , vol.30 , Issue.9 , pp. 728-736
    • Nicholson, L.V.B.1    Johnson, M.A.2    Bushby, K.M.D.3
  • 29
    • 0030668762 scopus 로고    scopus 로고
    • The membrane-cytoskeleton interface: The role of dystrophin and utrophin
    • Winder S. J., The membrane-cytoskeleton interface: the role of dystrophin and utrophin Journal of Muscle Research and Cell Motility 1997 18 6 617 629
    • (1997) Journal of Muscle Research and Cell Motility , vol.18 , Issue.6 , pp. 617-629
    • Winder, S.J.1
  • 30
    • 0038725634 scopus 로고    scopus 로고
    • Pharmacological strategies for muscular dystrophy
    • Khurana T. S., Davies K. E., Pharmacological strategies for muscular dystrophy Nature Reviews Drug Discovery 2003 2 5 379 390
    • (2003) Nature Reviews Drug Discovery , vol.2 , Issue.5 , pp. 379-390
    • Khurana, T.S.1    Davies, K.E.2
  • 31
    • 0027186337 scopus 로고
    • Dystrophin is phosphorylated by endogenous protein kinases
    • Luise M., Presotto C., Senter L., Dystrophin is phosphorylated by endogenous protein kinases Biochemical Journal 1993 293 1 243 247
    • (1993) Biochemical Journal , vol.293 , Issue.1 , pp. 243-247
    • Luise, M.1    Presotto, C.2    Senter, L.3
  • 32
    • 0028928013 scopus 로고
    • Alternate binding of actin and calmodulin to multiple sites on dystrophin
    • Jarrett H. W., Foster J. L., Alternate binding of actin and calmodulin to multiple sites on dystrophin Journal of Biological Chemistry 1995 270 10 5578 5586
    • (1995) Journal of Biological Chemistry , vol.270 , Issue.10 , pp. 5578-5586
    • Jarrett, H.W.1    Foster, J.L.2
  • 35
    • 0029000179 scopus 로고
    • Characterization of the recombinant C-terminal domain of dystrophin: Phosphorylation by calmodulin-dependent protein kinase II and dephosphorylation by type 2B protein phosphatase
    • Walsh M. P., Busaan J. L., Fraser E. D., Fu S. Y., Pato M. D., Michalak M., Characterization of the recombinant C-terminal domain of dystrophin: phosphorylation by calmodulin-dependent protein kinase II and dephosphorylation by type 2B protein phosphatase Biochemistry 1995 34 16 5561 5568
    • (1995) Biochemistry , vol.34 , Issue.16 , pp. 5561-5568
    • Walsh, M.P.1    Busaan, J.L.2    Fraser, E.D.3    Fu, S.Y.4    Pato, M.D.5    Michalak, M.6
  • 36
    • 0035190381 scopus 로고    scopus 로고
    • The dystrophin-glycoprotein complex, cellular signaling, and the regulation of cell survival in the muscular dystrophies
    • Rando T. A., The dystrophin-glycoprotein complex, cellular signaling, and the regulation of cell survival in the muscular dystrophies Muscle and Nerve 2001 24 12 1575 1594
    • (2001) Muscle and Nerve , vol.24 , Issue.12 , pp. 1575-1594
    • Rando, T.A.1
  • 37
    • 0032127263 scopus 로고    scopus 로고
    • The dystrophinopathies: An alternative to the structural hypothesis
    • Carlson C. G., The dystrophinopathies: an alternative to the structural hypothesis Neurobiology of Disease 1998 5 1 3 15
    • (1998) Neurobiology of Disease , vol.5 , Issue.1 , pp. 3-15
    • Carlson, C.G.1
  • 38
    • 0029877020 scopus 로고    scopus 로고
    • Dystrophin in the nervous system
    • Lidov H. G. W., Dystrophin in the nervous system Brain Pathology 1996 6 1 63 77
    • (1996) Brain Pathology , vol.6 , Issue.1 , pp. 63-77
    • Lidov, H.G.W.1
  • 39
    • 0034632180 scopus 로고    scopus 로고
    • Differential expression of utrophin and dystrophin in CNS neurons: An in situ hybridization and immunohistochemical study
    • Knuesel I., Bornhauser B. C., Zuellig R. A., Heller F., Schaub M. C., Fritschy J.-M., Differential expression of utrophin and dystrophin in CNS neurons: an in situ hybridization and immunohistochemical study Journal of Comparative Neurology 2000 422 4 594 611
    • (2000) Journal of Comparative Neurology , vol.422 , Issue.4 , pp. 594-611
    • Knuesel, I.1    Bornhauser, B.C.2    Zuellig, R.A.3    Heller, F.4    Schaub, M.C.5    Fritschy, J.-M.6
  • 40
    • 58849154498 scopus 로고    scopus 로고
    • A deficit of brain dystrophin impairs specific amygdala GABAergic transmission and enhances defensive behaviour in mice
    • Sekiguchi M., Zushida K., Yoshida M., A deficit of brain dystrophin impairs specific amygdala GABAergic transmission and enhances defensive behaviour in mice Brain 2009 132 1 124 135
    • (2009) Brain , vol.132 , Issue.1 , pp. 124-135
    • Sekiguchi, M.1    Zushida, K.2    Yoshida, M.3
  • 41
    • 0032805158 scopus 로고    scopus 로고
    • Effects of dystrophin isoforms on signal transduction through neural retina: Genotype-phenotype analysis of Duchenne muscular dystrophy mouse mutants
    • Pillers D.-A. M., Weleber R. G., Green D. G., Effects of dystrophin isoforms on signal transduction through neural retina: genotype-phenotype analysis of Duchenne muscular dystrophy mouse mutants Molecular Genetics and Metabolism 1999 66 2 100 110
    • (1999) Molecular Genetics and Metabolism , vol.66 , Issue.2 , pp. 100-110
    • Pillers, D.-A.M.1    Weleber, R.G.2    Green, D.G.3
  • 43
  • 44
    • 0028937525 scopus 로고
    • Dp140: A novel 140 kDa CNS transcript from the dystrophin locus
    • Lidov H. G. W., Selig S., Kunkel L. M., Dp140: a novel 140 kDa CNS transcript from the dystrophin locus Human Molecular Genetics 1995 4 3 329 335
    • (1995) Human Molecular Genetics , vol.4 , Issue.3 , pp. 329-335
    • Lidov, H.G.W.1    Selig, S.2    Kunkel, L.M.3
  • 45
    • 0027214837 scopus 로고
    • An alternative dystrophin transcript specific to peripheral nerve
    • Byers T. J., Lidov H. G. W., Kunkel L. M., An alternative dystrophin transcript specific to peripheral nerve Nature Genetics 1993 4 1 77 81
    • (1993) Nature Genetics , vol.4 , Issue.1 , pp. 77-81
    • Byers, T.J.1    Lidov, H.G.W.2    Kunkel, L.M.3
  • 47
    • 6344219969 scopus 로고    scopus 로고
    • The role of utrophin and Dp71 for assembly of different dystrophin-associated protein complexes (DPCs) in the choroid plexus and microvasculature of the brain
    • Haenggi T., Soontornmalai A., Schaub M. C., Fritschy J.-M., The role of utrophin and Dp71 for assembly of different dystrophin-associated protein complexes (DPCs) in the choroid plexus and microvasculature of the brain Neuroscience 2004 129 2 403 413
    • (2004) Neuroscience , vol.129 , Issue.2 , pp. 403-413
    • Haenggi, T.1    Soontornmalai, A.2    Schaub, M.C.3    Fritschy, J.-M.4
  • 48
    • 0028941158 scopus 로고
    • Specific expression of G-dystrophin (Dp71) in the brain
    • Gorecki D. C., Barnard E. A., Specific expression of G-dystrophin (Dp71) in the brain NeuroReport 1995 6 6 893 896
    • (1995) NeuroReport , vol.6 , Issue.6 , pp. 893-896
    • Gorecki, D.C.1    Barnard, E.A.2
  • 49
    • 0035086063 scopus 로고    scopus 로고
    • Subcellular localization of Dp71 dystrophin isoforms in cultured hippocampal neurons and forebrain astrocytes
    • Aleman V., Osorio B., Chavez O., Rendon A., Mornet D., Martinez D., Subcellular localization of Dp71 dystrophin isoforms in cultured hippocampal neurons and forebrain astrocytes Histochemistry and Cell Biology 2001 115 3 243 254
    • (2001) Histochemistry and Cell Biology , vol.115 , Issue.3 , pp. 243-254
    • Aleman, V.1    Osorio, B.2    Chavez, O.3    Rendon, A.4    Mornet, D.5    Martinez, D.6
  • 50
    • 0033230437 scopus 로고    scopus 로고
    • Different dystrophin-like complexes are expressed in neurons and glia
    • Blake D. J., Hawkes R., Benson M. A., Beesley P. W., Different dystrophin-like complexes are expressed in neurons and glia Journal of Cell Biology 1999 147 3 645 658
    • (1999) Journal of Cell Biology , vol.147 , Issue.3 , pp. 645-658
    • Blake, D.J.1    Hawkes, R.2    Benson, M.A.3    Beesley, P.W.4
  • 51
    • 74249107936 scopus 로고    scopus 로고
    • Role of mental retardation-associated dystrophin-gene product Dp71 in excitatory synapse organization, synaptic plasticity and behavioral functions
    • Daoud F., Candelario-Martinez A., Billard J. M., Role of mental retardation-associated dystrophin-gene product Dp71 in excitatory synapse organization, synaptic plasticity and behavioral functions PloS One 2009 4 8 e6574
    • (2009) PloS One , vol.4 , Issue.8 , pp. 6574
    • Daoud, F.1    Candelario-Martinez, A.2    Billard, J.M.3
  • 52
    • 0028837312 scopus 로고
    • The human dystrophin gene requires 16 hours to be transcribed and is cotranscriptionally spliced
    • Tennyson C. N., Klamut H. J., Worton R. G., The human dystrophin gene requires 16 hours to be transcribed and is cotranscriptionally spliced Nature Genetics 1995 9 2 184 190
    • (1995) Nature Genetics , vol.9 , Issue.2 , pp. 184-190
    • Tennyson, C.N.1    Klamut, H.J.2    Worton, R.G.3
  • 54
    • 0031260401 scopus 로고    scopus 로고
    • Dp140: Alternatively spliced isoforms in brain and kidney
    • Lidov H. G. W., Kunkel L. M., Dp140: alternatively spliced isoforms in brain and kidney Genomics 1997 45 1 132 139
    • (1997) Genomics , vol.45 , Issue.1 , pp. 132-139
    • Lidov, H.G.W.1    Kunkel, L.M.2
  • 55
    • 12444346407 scopus 로고    scopus 로고
    • Differential expression and subcellular distribution of dystrophin Dp71 isoforms during differentiation process
    • Marquez F. G., Cisneros B., Garcia F., Differential expression and subcellular distribution of dystrophin Dp71 isoforms during differentiation process Neuroscience 2003 118 4 957 966
    • (2003) Neuroscience , vol.118 , Issue.4 , pp. 957-966
    • Marquez, F.G.1    Cisneros, B.2    Garcia, F.3
  • 56
    • 0034162942 scopus 로고    scopus 로고
    • Expression and synthesis of alternatively spliced variants of Dp71 in adult human brain
    • Austin R. C., Morris G. E., Howard P. L., Klamut H. J., Ray P. N., Expression and synthesis of alternatively spliced variants of Dp71 in adult human brain Neuromuscular Disorders 2000 10 3 187 193
    • (2000) Neuromuscular Disorders , vol.10 , Issue.3 , pp. 187-193
    • Austin, R.C.1    Morris, G.E.2    Howard, P.L.3    Klamut, H.J.4    Ray, P.N.5
  • 57
    • 0037033057 scopus 로고    scopus 로고
    • Identification of Dp71 isoforms in the platelet membrane cytoskeleton: Potential role in thrombin-mediated platelet adhesion
    • Austin R. C., Fox J. E. B., Werstuck G. H., Identification of Dp71 isoforms in the platelet membrane cytoskeleton: potential role in thrombin-mediated platelet adhesion Journal of Biological Chemistry 2002 277 49 47106 47113
    • (2002) Journal of Biological Chemistry , vol.277 , Issue.49 , pp. 47106-47113
    • Austin, R.C.1    Fox, J.E.B.2    Werstuck, G.H.3
  • 58
    • 0030669534 scopus 로고    scopus 로고
    • A splice variant of Dp71 lacking the syntrophin binding site is expressed in early stages of human neural development
    • Ceccarini M., Rizzo G., Rosa G., Chelucci C., Macioce P., Petrucci T. C., A splice variant of Dp71 lacking the syntrophin binding site is expressed in early stages of human neural development Developmental Brain Research 1997 103 1 77 82
    • (1997) Developmental Brain Research , vol.103 , Issue.1 , pp. 77-82
    • Ceccarini, M.1    Rizzo, G.2    Rosa, G.3    Chelucci, C.4    MacIoce, P.5    Petrucci, T.C.6
  • 59
    • 33846991596 scopus 로고    scopus 로고
    • The dystrotelin, dystrophin and dystrobrevin superfamily: New paralogues and old isoforms
    • Jin H., Tan S., Hermanowski J., The dystrotelin, dystrophin and dystrobrevin superfamily: new paralogues and old isoforms BMC Genomics 2007 8, article 19
    • (2007) BMC Genomics , vol.819
    • Jin, H.1    Tan, S.2    Hermanowski, J.3
  • 60
    • 0024538824 scopus 로고
    • An autosomal transcript in skeletal muscle with homology to dystrophin
    • Love D. R., Hill D. F., Dickson G., An autosomal transcript in skeletal muscle with homology to dystrophin Nature 1989 339 6219 55 58
    • (1989) Nature , vol.339 , Issue.6219 , pp. 55-58
    • Love, D.R.1    Hill, D.F.2    Dickson, G.3
  • 61
    • 0026621049 scopus 로고
    • Primary structure of dystrophin-related protein
    • Tinsley J. M., Blake D. J., Roche A., Primary structure of dystrophin-related protein Nature 1992 360 6404 591 593
    • (1992) Nature , vol.360 , Issue.6404 , pp. 591-593
    • Tinsley, J.M.1    Blake, D.J.2    Roche, A.3
  • 62
    • 0025112892 scopus 로고
    • Localisation of a dystrophin-related autosomal gene to 6q24 in man, and to mouse chromosome 10 in the region of the dystrophia muscularis (dy) locus
    • Buckle V. J., Guenet J. L., Simon-Chazottes D., Love D. R., Davies K. E., Localisation of a dystrophin-related autosomal gene to 6q24 in man, and to mouse chromosome 10 in the region of the dystrophia muscularis (dy) locus Human Genetics 1990 85 3 324 326
    • (1990) Human Genetics , vol.85 , Issue.3 , pp. 324-326
    • Buckle, V.J.1    Guenet, J.L.2    Simon-Chazottes, D.3    Love, D.R.4    Davies, K.E.5
  • 64
  • 67
    • 0025932274 scopus 로고
    • Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle
    • Khurana T. S., Watkins S. C., Chafey P., Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle Neuromuscular Disorders 1991 1 3 185 194
    • (1991) Neuromuscular Disorders , vol.1 , Issue.3 , pp. 185-194
    • Khurana, T.S.1    Watkins, S.C.2    Chafey, P.3
  • 68
    • 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., Davies K. E., A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle Journal of Biological Chemistry 2002 277 47 45285 45290
    • (2002) Journal of Biological Chemistry , vol.277 , Issue.47 , pp. 45285-45290
    • Weir, A.P.1    Burton, E.A.2    Harrod, G.3    Davies, K.E.4
  • 69
    • 0027769345 scopus 로고
    • Utrophin: A potential replacement for dystrophin?
    • Tinsley J. M., Davies K. E., Utrophin: a potential replacement for dystrophin? Neuromuscular Disorders 1993 3 5-6 537 539
    • (1993) Neuromuscular Disorders , vol.3 , Issue.56 , pp. 537-539
    • Tinsley, J.M.1    Davies, K.E.2
  • 70
    • 0026094250 scopus 로고
    • Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle
    • Ohlendieck K., Ervasti J. M., Matsumura K., Kahl S. D., Leveille C. J., Campbell K. P., Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle Neuron 1991 7 3 499 508
    • (1991) Neuron , vol.7 , Issue.3 , pp. 499-508
    • Ohlendieck, K.1    Ervasti, J.M.2    Matsumura, K.3    Kahl, S.D.4    Leveille, C.J.5    Campbell, K.P.6
  • 71
    • 0031011395 scopus 로고    scopus 로고
    • Different utrophin and dystrophin properties related to their vascular smooth muscle distributions
    • Rivier F., Robert A., Hugon G., Mornet D., Different utrophin and dystrophin properties related to their vascular smooth muscle distributions FEBS Letters 1997 408 1 94 98
    • (1997) FEBS Letters , vol.408 , Issue.1 , pp. 94-98
    • Rivier, F.1    Robert, A.2    Hugon, G.3    Mornet, D.4
  • 72
    • 0028863572 scopus 로고
    • Dystrophin-related protein in the platelet membrane skeleton. Integrin- induced change in detergent-insolubility and cleavage by calpain in aggregating platelets
    • Earnest J. P., Santos G. F., Zuerbig S., Fox J. E. B., Dystrophin-related protein in the platelet membrane skeleton. Integrin- induced change in detergent-insolubility and cleavage by calpain in aggregating platelets Journal of Biological Chemistry 1995 270 45 27259 27265
    • (1995) Journal of Biological Chemistry , vol.270 , Issue.45 , pp. 27259-27265
    • Earnest, J.P.1    Santos, G.F.2    Zuerbig, S.3    Fox, J.E.B.4
  • 73
    • 0033066109 scopus 로고    scopus 로고
    • Characterization of dystrophin and utrophin diversity in the mouse
    • Lumeng C. N., Phelps S. F., Rafael J. A., Characterization of dystrophin and utrophin diversity in the mouse Human Molecular Genetics 1999 8 4 593 599
    • (1999) Human Molecular Genetics , vol.8 , Issue.4 , pp. 593-599
    • Lumeng, C.N.1    Phelps, S.F.2    Rafael, J.A.3
  • 74
    • 0027521883 scopus 로고
    • Differential expression of dystrophin, utrophin and dystrophin-associated proteins in peripheral nerve
    • Matsumura K., Yamada H., Shimizu T., Campbell K. P., Differential expression of dystrophin, utrophin and dystrophin-associated proteins in peripheral nerve FEBS Letters 1993 334 3 281 285
    • (1993) FEBS Letters , vol.334 , Issue.3 , pp. 281-285
    • Matsumura, K.1    Yamada, H.2    Shimizu, T.3    Campbell, K.P.4
  • 75
    • 0026781882 scopus 로고
    • The subcellular distribution of chromosome 6-encoded dystrophin-related protein in the brain
    • Khurana T. S., Watkins S. C., Kunkel L. M., The subcellular distribution of chromosome 6-encoded dystrophin-related protein in the brain Journal of Cell Biology 1992 119 2 357 366
    • (1992) Journal of Cell Biology , vol.119 , Issue.2 , pp. 357-366
    • Khurana, T.S.1    Watkins, S.C.2    Kunkel, L.M.3
  • 76
    • 0028297196 scopus 로고
    • Dystrophin-related protein is found in the central nervous system of mice at various developmental stages, especially at the postsynaptic membrane
    • Kamakura K., Tadano Y., Kawai M., Dystrophin-related protein is found in the central nervous system of mice at various developmental stages, especially at the postsynaptic membrane Journal of Neuroscience Research 1994 37 6 728 734
    • (1994) Journal of Neuroscience Research , vol.37 , Issue.6 , pp. 728-734
    • Kamakura, K.1    Tadano, Y.2    Kawai, M.3
  • 77
    • 45549098335 scopus 로고    scopus 로고
    • Kir4.1 and AQP4 associate with Dp71- and utrophin-DAPs complexes in specific and defined microdomains of Muller retinal glial cell membrane
    • Fort P. E., Sene A., Pannicke T., Kir4.1 and AQP4 associate with Dp71- and utrophin-DAPs complexes in specific and defined microdomains of Muller retinal glial cell membrane Glia 2008 56 6 597 610
    • (2008) Glia , vol.56 , Issue.6 , pp. 597-610
    • Fort, P.E.1    Sene, A.2    Pannicke, T.3
  • 78
    • 75949101674 scopus 로고    scopus 로고
    • Differential expression of utrophin-A and -B promoters in the central nervous system (CNS) of normal and dystrophic mdx mice
    • Baby S. M., Bogdanovich S., Willmann G., Basu U., Lozynska O., Khurana T. S., Differential expression of utrophin-A and -B promoters in the central nervous system (CNS) of normal and dystrophic mdx mice Brain Pathology 2010 20 2 323 342
    • (2010) Brain Pathology , vol.20 , Issue.2 , pp. 323-342
    • Baby, S.M.1    Bogdanovich, S.2    Willmann, G.3    Basu, U.4    Lozynska, O.5    Khurana, T.S.6
  • 80
    • 0032770342 scopus 로고    scopus 로고
    • Up71 and Up140, two novel transcripts of utrophin that are homologues of short forms of dystrophin
    • Wilson J., Putt W., Jimenez C., Edwards Y. H., Up71 and Up140, two novel transcripts of utrophin that are homologues of short forms of dystrophin Human Molecular Genetics 1999 8 7 1271 1278
    • (1999) Human Molecular Genetics , vol.8 , Issue.7 , pp. 1271-1278
    • Wilson, J.1    Putt, W.2    Jimenez, C.3    Edwards, Y.H.4
  • 81
    • 0028808198 scopus 로고
    • Re-evaluation of the distributions of dystrophin and utrophin in sciatic nerve
    • Fabbrizio E., Latouche J., Rivier F., Hugon G., Mornet D., Re-evaluation of the distributions of dystrophin and utrophin in sciatic nerve Biochemical Journal 1995 312 1 309 314
    • (1995) Biochemical Journal , vol.312 , Issue.1 , pp. 309-314
    • Fabbrizio, E.1    Latouche, J.2    Rivier, F.3    Hugon, G.4    Mornet, D.5
  • 83
    • 12244289646 scopus 로고    scopus 로고
    • A study of short utrophin isoforms in mice deficient for full-length utrophin
    • Jimenez-Mallebrera C., Davies K., Putt W., Edwards Y. H., A study of short utrophin isoforms in mice deficient for full-length utrophin Mammalian Genome 2003 14 1 47 60
    • (2003) Mammalian Genome , vol.14 , Issue.1 , pp. 47-60
    • Jimenez-Mallebrera, C.1    Davies, K.2    Putt, W.3    Edwards, Y.H.4
  • 84
    • 0030006540 scopus 로고    scopus 로고
    • Characterization of DRP2, a novel human dystrophin homologue
    • Roberts R. G., Freeman T. C., Kendall E., Characterization of DRP2, a novel human dystrophin homologue Nature Genetics 1996 13 2 223 226
    • (1996) Nature Genetics , vol.13 , Issue.2 , pp. 223-226
    • Roberts, R.G.1    Freeman, T.C.2    Kendall, E.3
  • 85
    • 0034532490 scopus 로고    scopus 로고
    • Association of dystrophin-related protein 2 (DRP2) with postsynaptic densities in rat brain
    • Roberts R. G., Sheng M., Association of dystrophin-related protein 2 (DRP2) with postsynaptic densities in rat brain Molecular and Cellular Neuroscience 2000 16 5 674 685
    • (2000) Molecular and Cellular Neuroscience , vol.16 , Issue.5 , pp. 674-685
    • Roberts, R.G.1    Sheng, M.2
  • 86
    • 0034968820 scopus 로고    scopus 로고
    • Specific disruption of a Schwann cell dystrophin-related protein complex in a demyelinating neuropathy
    • Sherman D. L., Fabrizi C., Gillespie C. Stewart, Brophy P. J., Specific disruption of a Schwann cell dystrophin-related protein complex in a demyelinating neuropathy Neuron 2001 30 3 677 687
    • (2001) Neuron , vol.30 , Issue.3 , pp. 677-687
    • Sherman, D.L.1    Fabrizi, C.2    Gillespie, C.S.3    Brophy, P.J.4
  • 88
    • 0030568868 scopus 로고    scopus 로고
    • Characterisation of the dystrophin-related protein utrophin in highly purified skeletal muscle sarcolemma vesicles
    • Ohlendieck K., Characterisation of the dystrophin-related protein utrophin in highly purified skeletal muscle sarcolemma vesicles Biochimica et Biophysica Acta 1996 1283 2 215 222
    • (1996) Biochimica et Biophysica Acta , vol.1283 , Issue.2 , pp. 215-222
    • Ohlendieck, K.1
  • 89
    • 0036560492 scopus 로고    scopus 로고
    • Syntrophins and dystrobrevins: Defining the dystrophin scaffold at synapses
    • Albrecht D. E., Froehner S. C., Syntrophins and dystrobrevins: defining the dystrophin scaffold at synapses NeuroSignals 2002 11 3 123 129
    • (2002) NeuroSignals , vol.11 , Issue.3 , pp. 123-129
    • Albrecht, D.E.1    Froehner, S.C.2
  • 90
    • 67449091712 scopus 로고    scopus 로고
    • The neurobiology of the dystrophin-associated glycoprotein complex
    • Waite A., Tinsley C. L., Locke M., Blake D. J., The neurobiology of the dystrophin-associated glycoprotein complex Annals of Medicine 2009 41 5 344 359
    • (2009) Annals of Medicine , vol.41 , Issue.5 , pp. 344-359
    • Waite, A.1    Tinsley, C.L.2    Locke, M.3    Blake, D.J.4
  • 91
    • 0030927063 scopus 로고    scopus 로고
    • Dystroglycan is essential for early embryonic development: Disruption of Reichert's membrane in Dag1-null mice
    • Williamson R. A., Henry M. D., Daniels K. J., Dystroglycan is essential for early embryonic development: disruption of Reichert's membrane in Dag1-null mice Human Molecular Genetics 1997 6 6 831 841
    • (1997) Human Molecular Genetics , vol.6 , Issue.6 , pp. 831-841
    • Williamson, R.A.1    Henry, M.D.2    Daniels, K.J.3
  • 92
    • 0037173629 scopus 로고    scopus 로고
    • Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy
    • Moore S. A., Saito F., Chen J., Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy Nature 2002 418 6896 422 425
    • (2002) Nature , vol.418 , Issue.6896 , pp. 422-425
    • Moore, S.A.1    Saito, F.2    Chen, J.3
  • 93
    • 1842453359 scopus 로고    scopus 로고
    • Defective glycosylation in congenital muscular dystrophies
    • DOI 10.1097/00019052-200404000-00020
    • Muntoni F., Brockington M., Torelli S., Brown S. C., Defective glycosylation in congenital muscular dystrophies Current Opinion in Neurology 2004 17 2 205 209 (Pubitemid 38446465)
    • (2004) Current Opinion in Neurology , vol.17 , Issue.2 , pp. 205-209
    • Muntoni, F.1    Brockington, M.2    Torelli, S.3    Brown, S.C.4
  • 94
    • 0030843272 scopus 로고    scopus 로고
    • Sequential roles of agrin, MuSK and rapsyn during neuromuscular junction formation
    • Glass D. J., Yancopoulos G. D., Sequential roles of agrin, MuSK and rapsyn during neuromuscular junction formation Current Opinion in Neurobiology 1997 7 3 379 384
    • (1997) Current Opinion in Neurobiology , vol.7 , Issue.3 , pp. 379-384
    • Glass, D.J.1    Yancopoulos, G.D.2
  • 95
    • 0033037910 scopus 로고    scopus 로고
    • Association of the dystroglycan complex isolated from bovine brain synaptosomes with proteins involved in signal transduction
    • Cavaldesi M., Macchia G., Barca S., Defilippi P., Tarone G., Petrucci T. C., Association of the dystroglycan complex isolated from bovine brain synaptosomes with proteins involved in signal transduction Journal of Neurochemistry 1999 72 4 1648 1655
    • (1999) Journal of Neurochemistry , vol.72 , Issue.4 , pp. 1648-1655
    • Cavaldesi, M.1    MacChia, G.2    Barca, S.3    Defilippi, P.4    Tarone, G.5    Petrucci, T.C.6
  • 96
    • 0035968170 scopus 로고    scopus 로고
    • Dysbindin, a a novel coiled-coil-containing protein that interacts with the dystrobrevins in muscle and brain
    • Benson M. A., Newey S. E., Martin-Rendon E., Hawkes R., Blake D. J., Dysbindin, a a novel coiled-coil-containing protein that interacts with the dystrobrevins in muscle and brain Journal of Biological Chemistry 2001 276 26 24232 24241
    • (2001) Journal of Biological Chemistry , vol.276 , Issue.26 , pp. 24232-24241
    • Benson, M.A.1    Newey, S.E.2    Martin-Rendon, E.3    Hawkes, R.4    Blake, D.J.5
  • 97
    • 33749563041 scopus 로고    scopus 로고
    • Dysbindin-1 is a synaptic and microtubular protein that binds brain snapin
    • Talbot K., Cho D.-S., Ong W.-Y., Dysbindin-1 is a synaptic and microtubular protein that binds brain snapin Human Molecular Genetics 2006 15 20 3041 3054
    • (2006) Human Molecular Genetics , vol.15 , Issue.20 , pp. 3041-3054
    • Talbot, K.1    Cho, D.-S.2    Ong, W.-Y.3
  • 99
    • 8044260252 scopus 로고    scopus 로고
    • Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice
    • Deconinck A. E., Potter A. C., Tinsley J. M., Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice Journal of Cell Biology 1997 136 4 883 894
    • (1997) Journal of Cell Biology , vol.136 , Issue.4 , pp. 883-894
    • Deconinck, A.E.1    Potter, A.C.2    Tinsley, J.M.3
  • 100
    • 0031036977 scopus 로고    scopus 로고
    • Subtle neuromuscular defects in utrophin-deficient mice
    • Grady R. M., Merlie J. P., Sanes J. R., Subtle neuromuscular defects in utrophin-deficient mice Journal of Cell Biology 1997 136 4 871 882
    • (1997) Journal of Cell Biology , vol.136 , Issue.4 , pp. 871-882
    • Grady, R.M.1    Merlie, J.P.2    Sanes, J.R.3
  • 101
    • 0034683669 scopus 로고    scopus 로고
    • Absence of -syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin
    • Adams M. E., Kramarcy N., Krall S. P., Absence of -syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin Journal of Cell Biology 2000 150 6 1385 1398
    • (2000) Journal of Cell Biology , vol.150 , Issue.6 , pp. 1385-1398
    • Adams, M.E.1    Kramarcy, N.2    Krall, S.P.3
  • 102
    • 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., Froehner S. C., The postsynaptic submembrane machinery at the neuromuscular junction: requirement for rapsyn and the utrophin/dystrophin-associated complex Brain Cell Biology 2003 32 58 709 726
    • (2003) Brain Cell Biology , vol.32 , Issue.58 , pp. 709-726
    • Banks, G.B.1    Fuhrer, C.2    Adams, M.E.3    Froehner, S.C.4
  • 103
    • 0035809195 scopus 로고    scopus 로고
    • The dystroglycan complex is necessary for stabilization of acetylcholine receptor clusters at neuromuscular junctions and formation of the synaptic basement membrane
    • Jacobson C., Cote P. D., Rossi S. G., Rotundo R. L., Carbonetto S., The dystroglycan complex is necessary for stabilization of acetylcholine receptor clusters at neuromuscular junctions and formation of the synaptic basement membrane Journal of Cell Biology 2001 153 3 435 450
    • (2001) Journal of Cell Biology , vol.153 , Issue.3 , pp. 435-450
    • Jacobson, C.1    Cote, P.D.2    Rossi, S.G.3    Rotundo, R.L.4    Carbonetto, S.5
  • 104
    • 0037071873 scopus 로고    scopus 로고
    • Neurotransmitter receptor dynamics studied in vivo by reversible photo-unbinding of fluorescent ligands
    • Akaaboune M., Grady R. M., Turney S., Sanes J. R., Lichtman J. W., Neurotransmitter receptor dynamics studied in vivo by reversible photo-unbinding of fluorescent ligands Neuron 2002 34 6 865 876
    • (2002) Neuron , vol.34 , Issue.6 , pp. 865-876
    • Akaaboune, M.1    Grady, R.M.2    Turney, S.3    Sanes, J.R.4    Lichtman, J.W.5
  • 105
    • 56149099711 scopus 로고    scopus 로고
    • Sarcospan reduces dystrophic pathology: Stabilization of the utrophin-glycoprotein complex
    • Peter A. K., Marshall J. L., Crosbie R. H., Sarcospan reduces dystrophic pathology: stabilization of the utrophin-glycoprotein complex Journal of Cell Biology 2008 183 3 419 427
    • (2008) Journal of Cell Biology , vol.183 , Issue.3 , pp. 419-427
    • Peter, A.K.1    Marshall, J.L.2    Crosbie, R.H.3
  • 106
    • 0030872016 scopus 로고    scopus 로고
    • Differential association of syntrophin pairs with the dystrophin complex
    • Peters M. F., Adams M. E., Froehner S. C., Differential association of syntrophin pairs with the dystrophin complex Journal of Cell Biology 1997 138 1 81 93
    • (1997) Journal of Cell Biology , vol.138 , Issue.1 , pp. 81-93
    • Peters, M.F.1    Adams, M.E.2    Froehner, S.C.3
  • 107
    • 0034039679 scopus 로고    scopus 로고
    • Syntrophin isoforms at the neuromuscular junction: Developmental time course and differential localization
    • Kramarcy N. R., Sealock R., Syntrophin isoforms at the neuromuscular junction: developmental time course and differential localization Molecular and Cellular Neurosciences 2000 15 3 262 274
    • (2000) Molecular and Cellular Neurosciences , vol.15 , Issue.3 , pp. 262-274
    • Kramarcy, N.R.1    Sealock, R.2
  • 108
    • 0028906307 scopus 로고
    • Mdx mice show progressive weakness and muscle deterioration with age
    • Pastoret C., Sebille A., mdx mice show progressive weakness and muscle deterioration with age Journal of the Neurological Sciences 1995 129 2 97 105
    • (1995) Journal of the Neurological Sciences , vol.129 , Issue.2 , pp. 97-105
    • Pastoret, C.1    Sebille, A.2
  • 109
    • 33644996933 scopus 로고    scopus 로고
    • Cerebellar synaptic defects and abnormal motor behavior in mice lacking - And -dystrobrevin
    • Grady R. M., Wozniak D. F., Ohlemiller K. K., Sanes J. R., Cerebellar synaptic defects and abnormal motor behavior in mice lacking -and -dystrobrevin Journal of Neuroscience 2006 26 11 2841 2851
    • (2006) Journal of Neuroscience , vol.26 , Issue.11 , pp. 2841-2851
    • Grady, R.M.1    Wozniak, D.F.2    Ohlemiller, K.K.3    Sanes, J.R.4
  • 110
    • 4444324797 scopus 로고    scopus 로고
    • Impaired long-term spatial and recognition memory and enhanced CA1 hippocampal LTP in the dystrophin-deficient Dmdmdx mouse
    • Vaillend C., Billard J.-M., Laroche S., Impaired long-term spatial and recognition memory and enhanced CA1 hippocampal LTP in the dystrophin-deficient Dmdmdx mouse Neurobiology of Disease 2004 17 1 10 20
    • (2004) Neurobiology of Disease , vol.17 , Issue.1 , pp. 10-20
    • Vaillend, C.1    Billard, J.-M.2    Laroche, S.3
  • 111
    • 0030604662 scopus 로고    scopus 로고
    • Spatial learning and hippocampal long-term potentiation are not impaired in mdx mice
    • Sesay A. K., Errington M. L., Levita L., Bliss T. V. P., Spatial learning and hippocampal long-term potentiation are not impaired in mdx mice Neuroscience Letters 1996 211 3 207 210
    • (1996) Neuroscience Letters , vol.211 , Issue.3 , pp. 207-210
    • Sesay, A.K.1    Errington, M.L.2    Levita, L.3    Bliss, T.V.P.4
  • 112
    • 0031812738 scopus 로고    scopus 로고
    • Spatial discrimination learning and CA1 hippocampal synaptic plasticity in mdx and mdx(3cv) mice lacking dystrophin gene products
    • Vaillend C., Billard J.-M., Claudepierre T., Rendon A., Dutar P., Ungerer A., Spatial discrimination learning and CA1 hippocampal synaptic plasticity in mdx and mdx(3cv) mice lacking dystrophin gene products Neuroscience 1998 86 1 53 66
    • (1998) Neuroscience , vol.86 , Issue.1 , pp. 53-66
    • Vaillend, C.1    Billard, J.-M.2    Claudepierre, T.3    Rendon, A.4    Dutar, P.5    Ungerer, A.6
  • 113
    • 0026039523 scopus 로고
    • Passive avoidance behaviour deficit in the mdx mouse
    • Muntoni F., Mateddu A., Serra G., Passive avoidance behaviour deficit in the mdx mouse Neuromuscular Disorders 1991 1 2 121 123
    • (1991) Neuromuscular Disorders , vol.1 , Issue.2 , pp. 121-123
    • Muntoni, F.1    Mateddu, A.2    Serra, G.3
  • 114
    • 0028834843 scopus 로고
    • Influence of dystrophin-gene mutation on mdx mouse behavior. I. Retention deficits at long delays in spontaneous alternation and bar-pressing tasks
    • Vaillend C., Rendon A., Misslin R., Ungerer A., Influence of dystrophin-gene mutation on mdx mouse behavior. I. Retention deficits at long delays in spontaneous alternation and bar-pressing tasks Behavior Genetics 1995 25 6 569 579
    • (1995) Behavior Genetics , vol.25 , Issue.6 , pp. 569-579
    • Vaillend, C.1    Rendon, A.2    Misslin, R.3    Ungerer, A.4
  • 115
    • 0033167970 scopus 로고    scopus 로고
    • Facilitated NMDA receptor-mediated synaptic plasticity in the hippocampal CA1 area of dystrophin-deficient mice
    • Vaillend C., Ungerer A., Billard J.-M., Facilitated NMDA receptor-mediated synaptic plasticity in the hippocampal CA1 area of dystrophin-deficient mice Synapse 1999 33 1 59 70
    • (1999) Synapse , vol.33 , Issue.1 , pp. 59-70
    • Vaillend, C.1    Ungerer, A.2    Billard, J.-M.3
  • 116
    • 0033005157 scopus 로고    scopus 로고
    • Behavioral characterization of mdx3cv mice deficient in C-terminal dystrophins
    • Vaillend C., Ungerer A., Behavioral characterization of mdx3cv mice deficient in C-terminal dystrophins Neuromuscular Disorders 1999 9 5 296 304
    • (1999) Neuromuscular Disorders , vol.9 , Issue.5 , pp. 296-304
    • Vaillend, C.1    Ungerer, A.2
  • 118
    • 0037467614 scopus 로고    scopus 로고
    • Cortical and brainstem neurons containing calcium-binding proteins in a murine model of Duchenne's muscular dystrophy: Selective changes in the sensorimotor cortex
    • Carretta D., Santarelli M., Vanni D., Cortical and brainstem neurons containing calcium-binding proteins in a murine model of Duchenne's muscular dystrophy: selective changes in the sensorimotor cortex Journal of Comparative Neurology 2003 456 1 48 59
    • (2003) Journal of Comparative Neurology , vol.456 , Issue.1 , pp. 48-59
    • Carretta, D.1    Santarelli, M.2    Vanni, D.3
  • 119
    • 67651115700 scopus 로고    scopus 로고
    • Reorganization of inhibitory synapses and increased PSD length of perforated excitatory synapses in hippocampal area CA1 of dystrophin-deficient mdx mice
    • Miranda R., Sebrie C., Degrouard J., Reorganization of inhibitory synapses and increased PSD length of perforated excitatory synapses in hippocampal area CA1 of dystrophin-deficient mdx mice Cerebral Cortex 2009 19 4 876 888
    • (2009) Cerebral Cortex , vol.19 , Issue.4 , pp. 876-888
    • Miranda, R.1    Sebrie, C.2    Degrouard, J.3
  • 120
    • 4043157672 scopus 로고    scopus 로고
    • Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice
    • Anderson J. L., Head S. I., Morley J. W., Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice Brain Research 2004 1019 1-2 289 292
    • (2004) Brain Research , vol.1019 , Issue.12 , pp. 289-292
    • Anderson, J.L.1    Head, S.I.2    Morley, J.W.3
  • 121
    • 77649155250 scopus 로고    scopus 로고
    • Enhanced homosynaptic LTD in cerebellar Purkinje cells of the dystrophic mdx mouse
    • Anderson J. L., Morley J. W., Head S. I., Enhanced homosynaptic LTD in cerebellar Purkinje cells of the dystrophic mdx mouse Muscle and Nerve 2010 41 3 329 334
    • (2010) Muscle and Nerve , vol.41 , Issue.3 , pp. 329-334
    • Anderson, J.L.1    Morley, J.W.2    Head, S.I.3
  • 122
    • 0034071559 scopus 로고    scopus 로고
    • A role for nitric oxide in muscle repair: Nitric oxide-mediated activation of muscle satellite cells
    • Anderson J. E., A role for nitric oxide in muscle repair: nitric oxide-mediated activation of muscle satellite cells Molecular Biology of the Cell 2000 11 5 1859 1874
    • (2000) Molecular Biology of the Cell , vol.11 , Issue.5 , pp. 1859-1874
    • Anderson, J.E.1
  • 123
    • 33745728934 scopus 로고    scopus 로고
    • Cognitive impairment in neuromuscular disorders
    • D'Angelo M. G., Bresolin N., Cognitive impairment in neuromuscular disorders Muscle and Nerve 2006 34 1 16 33
    • (2006) Muscle and Nerve , vol.34 , Issue.1 , pp. 16-33
    • D'Angelo, M.G.1    Bresolin, N.2
  • 124
    • 0037096375 scopus 로고    scopus 로고
    • GABAergic terminals are required for postsynaptic clustering of dystrophin but not of GABAA receptors and gephyrin
    • Brunig I., Suter A., Knuesel I., Luscher B., Fritschy J.-M., GABAergic terminals are required for postsynaptic clustering of dystrophin but not of GABAA receptors and gephyrin Journal of Neuroscience 2002 22 12 4805 4813
    • (2002) Journal of Neuroscience , vol.22 , Issue.12 , pp. 4805-4813
    • Brunig, I.1    Suter, A.2    Knuesel, I.3    Luscher, B.4    Fritschy, J.-M.5
  • 125
    • 0036616948 scopus 로고    scopus 로고
    • Dystroglycan is selectively associated with inhibitory GABAergic synapses but is dispensable for their differentiation
    • Levi S., Grady R. M., Henry M. D., Campbell K. P., Sanes J. R., Craig A. M., Dystroglycan is selectively associated with inhibitory GABAergic synapses but is dispensable for their differentiation Journal of Neuroscience 2002 22 11 4274 4285
    • (2002) Journal of Neuroscience , vol.22 , Issue.11 , pp. 4274-4285
    • Levi, S.1    Grady, R.M.2    Henry, M.D.3    Campbell, K.P.4    Sanes, J.R.5    Craig, A.M.6
  • 127
    • 0042067882 scopus 로고    scopus 로고
    • Pre- and post-synaptic mechanisms regulating the clustering of type A -aminobutyric acid receptors (GABAA receptors)
    • Fritschy J.-M., Schweizer C., Brunig I., Luscher B., Pre- and post-synaptic mechanisms regulating the clustering of type A -aminobutyric acid receptors (GABAA receptors) Biochemical Society Transactions 2003 31 4 889 892
    • (2003) Biochemical Society Transactions , vol.31 , Issue.4 , pp. 889-892
    • Fritschy, J.-M.1    Schweizer, C.2    Brunig, I.3    Luscher, B.4
  • 128
    • 33846867244 scopus 로고    scopus 로고
    • Neurexin-neuroligin signaling in synapse development
    • Craig A. M., Kang Y., Neurexin-neuroligin signaling in synapse development Current Opinion in Neurobiology 2007 17 1 43 52
    • (2007) Current Opinion in Neurobiology , vol.17 , Issue.1 , pp. 43-52
    • Craig, A.M.1    Kang, Y.2
  • 129
    • 0035063257 scopus 로고    scopus 로고
    • Alterations in dystrophin and utrophin expression parallel the reorganization of GABAergic synapses in a mouse model of temporal lobe epilepsy
    • Knuesel I., Zuellig R. A., Schaub M. C., Fritschy J.-M., Alterations in dystrophin and utrophin expression parallel the reorganization of GABAergic synapses in a mouse model of temporal lobe epilepsy European Journal of Neuroscience 2001 13 6 1113 1124
    • (2001) European Journal of Neuroscience , vol.13 , Issue.6 , pp. 1113-1124
    • Knuesel, I.1    Zuellig, R.A.2    Schaub, M.C.3    Fritschy, J.-M.4
  • 130
    • 0142102393 scopus 로고    scopus 로고
    • Altered inhibitory input to Purkinje cells of dystrophin-deficient mice
    • Anderson J. L., Head S. I., Morley J. W., Altered inhibitory input to Purkinje cells of dystrophin-deficient mice Brain Research 2003 982 2 280 283
    • (2003) Brain Research , vol.982 , Issue.2 , pp. 280-283
    • Anderson, J.L.1    Head, S.I.2    Morley, J.W.3
  • 131
    • 0036954395 scopus 로고    scopus 로고
    • Facilitated CA1 hippocampal synaptic plasticity in dystrophin-deficient mice: Role of GABAA receptors?
    • Vaillend C., Billard J.-M., Facilitated CA1 hippocampal synaptic plasticity in dystrophin-deficient mice: role of GABAA receptors? Hippocampus 2002 12 6 713 717
    • (2002) Hippocampus , vol.12 , Issue.6 , pp. 713-717
    • Vaillend, C.1    Billard, J.-M.2
  • 132
    • 38149137177 scopus 로고    scopus 로고
    • GABA A receptor expression and inhibitory post-synaptic currents in cerebellar Purkinje cells in dystrophin-deficient mdx mice
    • Kueh S. L. L., Head S. I., Morley J. W., GABA A receptor expression and inhibitory post-synaptic currents in cerebellar Purkinje cells in dystrophin-deficient mdx mice Clinical and Experimental Pharmacology and Physiology 2008 35 2 207 210
    • (2008) Clinical and Experimental Pharmacology and Physiology , vol.35 , Issue.2 , pp. 207-210
    • Kueh, S.L.L.1    Head, S.I.2    Morley, J.W.3
  • 133
    • 41549111538 scopus 로고    scopus 로고
    • GABAergic miniature spontaneous activity is increased in the CA1 hippocampal region of dystrophic mdx mice
    • Graciotti L., Minelli A., Minciacchi D., Procopio A., Fulgenzi G., GABAergic miniature spontaneous activity is increased in the CA1 hippocampal region of dystrophic mdx mice Neuromuscular Disorders 2008 18 3 220 226
    • (2008) Neuromuscular Disorders , vol.18 , Issue.3 , pp. 220-226
    • Graciotti, L.1    Minelli, A.2    Minciacchi, D.3    Procopio, A.4    Fulgenzi, G.5
  • 134
    • 71449105214 scopus 로고    scopus 로고
    • Parvalbumin-positive GABAergic interneurons are increased in the dorsal hippocampus of the dystrophic mdx mouse
    • Del Tongo C., Carretta D., Fulgenzi G., Catini C., Minciacchi D., Parvalbumin-positive GABAergic interneurons are increased in the dorsal hippocampus of the dystrophic mdx mouse Acta Neuropathologica 2009 118 6 803 812
    • (2009) Acta Neuropathologica , vol.118 , Issue.6 , pp. 803-812
    • Del Tongo, C.1    Carretta, D.2    Fulgenzi, G.3    Catini, C.4    Minciacchi, D.5
  • 135
    • 0344839040 scopus 로고    scopus 로고
    • Abnormal kainic acid receptor density and reduced seizure susceptibility in dystrophin-deficient mdx mice
    • Yoshihara Y., Onodera H., Iinuma K., Itoyama Y., Abnormal kainic acid receptor density and reduced seizure susceptibility in dystrophin-deficient mdx mice Neuroscience 2003 117 2 391 395
    • (2003) Neuroscience , vol.117 , Issue.2 , pp. 391-395
    • Yoshihara, Y.1    Onodera, H.2    Iinuma, K.3    Itoyama, Y.4
  • 137
    • 0037007978 scopus 로고    scopus 로고
    • Genetically dystrophic mdx/mdx mice exhibit decreased response to nicotine in passive avoidance
    • Coccurello R., Castellano C., Paggi P., Mele A., Oliverio A., Genetically dystrophic mdx/mdx mice exhibit decreased response to nicotine in passive avoidance NeuroReport 2002 13 9 1219 1222
    • (2002) NeuroReport , vol.13 , Issue.9 , pp. 1219-1222
    • Coccurello, R.1    Castellano, C.2    Paggi, P.3    Mele, A.4    Oliverio, A.5
  • 138
    • 0036315609 scopus 로고    scopus 로고
    • Dystrophin stabilizes 3- but not 7-containing nicotinic acetylcholine receptor subtypes at the postsynaptic apparatus in the mouse superior cervical ganglion
    • Del Signore A., Gotti C., De Stefano M. E., Moretti M., Paggi P., Dystrophin stabilizes 3- but not 7-containing nicotinic acetylcholine receptor subtypes at the postsynaptic apparatus in the mouse superior cervical ganglion Neurobiology of Disease 2002 10 1 54 66
    • (2002) Neurobiology of Disease , vol.10 , Issue.1 , pp. 54-66
    • Del Signore, A.1    Gotti, C.2    De Stefano, M.E.3    Moretti, M.4    Paggi, P.5
  • 139
    • 0038407772 scopus 로고    scopus 로고
    • Severe alterations of endothelial and glial cells in the blood-brain barrier of dystrophic mdx mice
    • Nico B., Frigeri A., Nicchia G. P., Severe alterations of endothelial and glial cells in the blood-brain barrier of dystrophic mdx mice Glia 2003 42 3 235 251
    • (2003) Glia , vol.42 , Issue.3 , pp. 235-251
    • Nico, B.1    Frigeri, A.2    Nicchia, G.P.3
  • 140
    • 2142814283 scopus 로고    scopus 로고
    • Altered blood-brain barrier development in dystrophic MDX mice
    • Nico B., Nicchia G. P., Frigeri A., Altered blood-brain barrier development in dystrophic MDX mice Neuroscience 2004 125 4 921 935
    • (2004) Neuroscience , vol.125 , Issue.4 , pp. 921-935
    • Nico, B.1    Nicchia, G.P.2    Frigeri, A.3
  • 141
    • 33646862798 scopus 로고    scopus 로고
    • Increased matrix-metalloproteinase-2 and matrix-metalloproteinase-9 expression in the brain of dystrophic mdx mouse
    • Nico B., Corsi P., Ria R., Increased matrix-metalloproteinase-2 and matrix-metalloproteinase-9 expression in the brain of dystrophic mdx mouse Neuroscience 2006 140 3 835 848
    • (2006) Neuroscience , vol.140 , Issue.3 , pp. 835-848
    • Nico, B.1    Corsi, P.2    Ria, R.3
  • 142
    • 35148829423 scopus 로고    scopus 로고
    • HIF activation and VEGF overexpression are coupled with ZO-1 up-phosphorylation in the brain of dystrophic MDX mouse
    • Nico B., Mangieri D., Crivellato E., HIF activation and VEGF overexpression are coupled with ZO-1 up-phosphorylation in the brain of dystrophic MDX mouse Brain Pathology 2007 17 4 399 406
    • (2007) Brain Pathology , vol.17 , Issue.4 , pp. 399-406
    • Nico, B.1    Mangieri, D.2    Crivellato, E.3
  • 143
    • 0025317772 scopus 로고
    • Decline in respiratory function and experience with long-term assisted ventilation in advanced Duchenne's muscular dystrophy
    • Baydur A., Gilgoff I., Prentice W., Carlson M., Fischer D. A., Decline in respiratory function and experience with long-term assisted ventilation in advanced Duchenne's muscular dystrophy Chest 1990 97 4 884 889
    • (1990) Chest , vol.97 , Issue.4 , pp. 884-889
    • Baydur, A.1    Gilgoff, I.2    Prentice, W.3    Carlson, M.4    Fischer, D.A.5
  • 144
    • 0032952705 scopus 로고    scopus 로고
    • Targeted inactivation of Dp71, the major non-muscle product of the DMD gene: Differential activity of the Dp71 promoter during development
    • Sarig R., Mezger-Lallemand V., Gitelman I., Targeted inactivation of Dp71, the major non-muscle product of the DMD gene: differential activity of the Dp71 promoter during development Human Molecular Genetics 1999 8 1 1 10
    • (1999) Human Molecular Genetics , vol.8 , Issue.1 , pp. 1-10
    • Sarig, R.1    Mezger-Lallemand, V.2    Gitelman, I.3
  • 146
    • 2442638032 scopus 로고    scopus 로고
    • Alpha-syntrophin deletion removes the perivascular but not endothelial pool of aquaporin-4 at the blood-brain barrier and delays the development of brain edema in an experimental model of acute hyponatremia
    • Amiry-Moghaddam M., Xue R., Haug F. M., Alpha-syntrophin deletion removes the perivascular but not endothelial pool of aquaporin-4 at the blood-brain barrier and delays the development of brain edema in an experimental model of acute hyponatremia FASEB Journal 2004 18 3 542 544
    • (2004) FASEB Journal , vol.18 , Issue.3 , pp. 542-544
    • Amiry-Moghaddam, M.1    Xue, R.2    Haug, F.M.3
  • 148
    • 46749120689 scopus 로고    scopus 로고
    • Dystrophin-dependent and -independent AQP4 pools are expressed in the mouse brain
    • Nicchia G. P., Rossi A., Nudel U., Svelto M., Frigeri A., Dystrophin-dependent and -independent AQP4 pools are expressed in the mouse brain Glia 2008 56 8 869 876
    • (2008) Glia , vol.56 , Issue.8 , pp. 869-876
    • Nicchia, G.P.1    Rossi, A.2    Nudel, U.3    Svelto, M.4    Frigeri, A.5
  • 149
    • 6344284791 scopus 로고    scopus 로고
    • Differential assembly of inwardly rectifying K+ channel subunits, Kir4.1 and Kir5.1, in brain astrocytes
    • Hibino H., Fujita A., Iwai K., Yamada M., Kurachi Y., Differential assembly of inwardly rectifying K+ channel subunits, Kir4.1 and Kir5.1, in brain astrocytes Journal of Biological Chemistry 2004 279 42 44065 44073
    • (2004) Journal of Biological Chemistry , vol.279 , Issue.42 , pp. 44065-44073
    • Hibino, H.1    Fujita, A.2    Iwai, K.3    Yamada, M.4    Kurachi, Y.5
  • 150
    • 3142580944 scopus 로고    scopus 로고
    • The potassium channel Kir4.1 associates with the dystrophin-glycoprotein complex via -syntrophin in glia
    • Connors N. C., Adams M. E., Froehner S. C., Kofuji P., The potassium channel Kir4.1 associates with the dystrophin-glycoprotein complex via -syntrophin in glia Journal of Biological Chemistry 2004 279 27 28387 28392
    • (2004) Journal of Biological Chemistry , vol.279 , Issue.27 , pp. 28387-28392
    • Connors, N.C.1    Adams, M.E.2    Froehner, S.C.3    Kofuji, P.4
  • 151
    • 3943068322 scopus 로고    scopus 로고
    • Laminin-induced aggregation of the inwardly rectifying potassium channel, Kir4.1, and the water-permeable channel, AQP4, via a dystroglycan-containing complex in astrocytes
    • Guadagno E., Moukhles H., Laminin-induced aggregation of the inwardly rectifying potassium channel, Kir4.1, and the water-permeable channel, AQP4, via a dystroglycan-containing complex in astrocytes Glia 2004 47 2 138 149
    • (2004) Glia , vol.47 , Issue.2 , pp. 138-149
    • Guadagno, E.1    Moukhles, H.2
  • 152
    • 70350238177 scopus 로고    scopus 로고
    • Functional implication of Dp71 in osmoregulation and vascular permeability of the retina
    • ARTICLE E7329
    • Sene A., Tadayoni R., Pannicke T., Functional implication of Dp71 in osmoregulation and vascular permeability of the retina PloS One 2009 4 10, article e7329
    • (2009) PloS One , vol.4 , pp. 10
    • Sene, A.1    Tadayoni, R.2    Pannicke, T.3
  • 153
    • 10744227341 scopus 로고    scopus 로고
    • Targeted inactivation of dystrophin gene product Dp71: Phenotypic impact in mouse retina
    • Dalloz C., Sarig R., Fort P., Targeted inactivation of dystrophin gene product Dp71: phenotypic impact in mouse retina Human Molecular Genetics 2003 12 13 1543 1554
    • (2003) Human Molecular Genetics , vol.12 , Issue.13 , pp. 1543-1554
    • Dalloz, C.1    Sarig, R.2    Fort, P.3
  • 154
  • 155
    • 67349162678 scopus 로고    scopus 로고
    • AQP4 gene deletion in mice does not alter blood-brain barrier integrity or brain morphology
    • Saadoun S., Tait M. J., Reza A., AQP4 gene deletion in mice does not alter blood-brain barrier integrity or brain morphology Neuroscience 2009 161 3 764 772
    • (2009) Neuroscience , vol.161 , Issue.3 , pp. 764-772
    • Saadoun, S.1    Tait, M.J.2    Reza, A.3
  • 156
    • 0345515991 scopus 로고    scopus 로고
    • Kainate-evoked changes in dystrophin messenger RNA levels in the rat hippocampus
    • Gorecki D. C., Lukasiuk K., Szklarczyk A., Kaczmarek L., Kainate-evoked changes in dystrophin messenger RNA levels in the rat hippocampus Neuroscience 1998 84 2 467 477
    • (1998) Neuroscience , vol.84 , Issue.2 , pp. 467-477
    • Gorecki, D.C.1    Lukasiuk, K.2    Szklarczyk, A.3    Kaczmarek, L.4
  • 157
    • 0036132661 scopus 로고    scopus 로고
    • Expression of dystrophin-associated proteins during neuronal differentiation of P19 embryonal carcinoma cells
    • Ceccarini M., Macioce P., Panetta B., Petrucci T. C., Expression of dystrophin-associated proteins during neuronal differentiation of P19 embryonal carcinoma cells Neuromuscular Disorders 2002 12 1 36 48
    • (2002) Neuromuscular Disorders , vol.12 , Issue.1 , pp. 36-48
    • Ceccarini, M.1    MacIoce, P.2    Panetta, B.3    Petrucci, T.C.4
  • 158
    • 33748743451 scopus 로고    scopus 로고
    • Dystrophin Dp71f associates with the 1-integrin adhesion complex to modulate PC12 cell adhesion
    • Cerna J., Cerecedo D., Ortega A., Dystrophin Dp71f associates with the 1-integrin adhesion complex to modulate PC12 cell adhesion Journal of Molecular Biology 2006 362 5 954 965
    • (2006) Journal of Molecular Biology , vol.362 , Issue.5 , pp. 954-965
    • Cerna, J.1    Cerecedo, D.2    Ortega, A.3
  • 159
    • 0030848969 scopus 로고    scopus 로고
    • Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy
    • Deconinck A. E., Rafael J. A., Skinner J. A., Utrophin-dystrophin- deficient mice as a model for Duchenne muscular dystrophy Cell 1997 90 4 717 727
    • (1997) Cell , vol.90 , Issue.4 , pp. 717-727
    • Deconinck, A.E.1    Rafael, J.A.2    Skinner, J.A.3
  • 160
    • 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., Sanest J. R., Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy Cell 1997 90 4 729 738
    • (1997) Cell , vol.90 , Issue.4 , pp. 729-738
    • Grady, R.M.1    Teng, H.2    Nichol, M.C.3    Cunningham, J.C.4    Wilkinson, R.S.5    Sanest, J.R.6
  • 161
    • 0036460279 scopus 로고    scopus 로고
    • Increased vulnerability to kainate-induced seizures in utrophin-knockout mice
    • Knuesel I., Riban V., Zuellig R. A., Increased vulnerability to kainate-induced seizures in utrophin-knockout mice European Journal of Neuroscience 2002 15 9 1474 1484
    • (2002) European Journal of Neuroscience , vol.15 , Issue.9 , pp. 1474-1484
    • Knuesel, I.1    Riban, V.2    Zuellig, R.A.3
  • 162
    • 2342594385 scopus 로고    scopus 로고
    • Brain dystrophin-glycoprotein complex: Persistent expression of beta-dystroglycan, impaired oligomerization of Dp71 and up-regulation of utrophins in animal models of muscular dystrophy
    • Culligan K., Glover L., Dowling P., Ohlendieck K., Brain dystrophin-glycoprotein complex: persistent expression of beta-dystroglycan, impaired oligomerization of Dp71 and up-regulation of utrophins in animal models of muscular dystrophy BMC Cell Biology 2001 2, article 2
    • (2001) BMC Cell Biology , vol.22
    • Culligan, K.1    Glover, L.2    Dowling, P.3    Ohlendieck, K.4
  • 163
    • 31644445575 scopus 로고    scopus 로고
    • Pathological pattern of Mdx mice diaphragm correlates with gradual expression of the short utrophin isoform Up71
    • Hnia K., Tuffery-Giraud S., Vermaelen M., Pathological pattern of Mdx mice diaphragm correlates with gradual expression of the short utrophin isoform Up71 Biochimica et Biophysica Acta 2006 1762 3 362 372
    • (2006) Biochimica et Biophysica Acta , vol.1762 , Issue.3 , pp. 362-372
    • Hnia, K.1    Tuffery-Giraud, S.2    Vermaelen, M.3
  • 164
    • 1542344795 scopus 로고    scopus 로고
    • Therapeutics for Duchenne muscular dystrophy: Current approaches and future directions
    • Bogdanovich S., Perkins K. J., Krag T. O. B., Khurana T. S., Therapeutics for Duchenne muscular dystrophy: current approaches and future directions Journal of Molecular Medicine 2004 82 2 102 115
    • (2004) Journal of Molecular Medicine , vol.82 , Issue.2 , pp. 102-115
    • Bogdanovich, S.1    Perkins, K.J.2    Krag, T.O.B.3    Khurana, T.S.4
  • 166
    • 0033812726 scopus 로고    scopus 로고
    • A multicenter, double-blind, randomized trial of deflazacort versus prednisone in duchenne muscular dystrophy
    • Bonifati M. D., Ruzza G., Bonometto P., A multicenter, double-blind, randomized trial of deflazacort versus prednisone in duchenne muscular dystrophy Muscle and Nerve 2000 23 9 1344 1347
    • (2000) Muscle and Nerve , vol.23 , Issue.9 , pp. 1344-1347
    • Bonifati, M.D.1    Ruzza, G.2    Bonometto, P.3
  • 167
    • 0024540879 scopus 로고
    • Conversion of mdx myofibres from dystrophin-negative to -positive by injection of normal myoblasts
    • Partridge T. A., Morgan J. E., Coulton G. R., Hoffman E. P., Kunkel L. M., Conversion of mdx myofibres from dystrophin-negative to -positive by injection of normal myoblasts Nature 1989 337 6203 176 179
    • (1989) Nature , vol.337 , Issue.6203 , pp. 176-179
    • Partridge, T.A.1    Morgan, J.E.2    Coulton, G.R.3    Hoffman, E.P.4    Kunkel, L.M.5
  • 168
    • 34247236574 scopus 로고    scopus 로고
    • Stem and progenitor cells in skeletal muscle development, maintenance, and therapy
    • Pault B., Rudnicki M., Torrente Y., Stem and progenitor cells in skeletal muscle development, maintenance, and therapy Molecular Therapy 2007 15 5 867 877
    • (2007) Molecular Therapy , vol.15 , Issue.5 , pp. 867-877
    • Pault, B.1    Rudnicki, M.2    Torrente, Y.3
  • 169
    • 0036736786 scopus 로고    scopus 로고
    • Long-term persistence of donor nuclei in a Duchenne muscular dystrophy patient receiving bone marrow transplantation
    • Gussoni E., Bennett R. R., Muskiewicz K. R., Long-term persistence of donor nuclei in a Duchenne muscular dystrophy patient receiving bone marrow transplantation Journal of Clinical Investigation 2002 110 6 807 814
    • (2002) Journal of Clinical Investigation , vol.110 , Issue.6 , pp. 807-814
    • Gussoni, E.1    Bennett, R.R.2    Muskiewicz, K.R.3
  • 170
    • 0034622814 scopus 로고    scopus 로고
    • Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy (mdx) mice
    • Ebihara S., Guibinga G.-H., Gilbert R., Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy (mdx) mice Physiological Genomics 2000 3 3 133 144
    • (2000) Physiological Genomics , vol.3 , Issue.3 , pp. 133-144
    • Ebihara, S.1    Guibinga, G.-H.2    Gilbert, R.3
  • 173
    • 0030605212 scopus 로고    scopus 로고
    • Utrophin to the rescue
    • Campbell K. P., Crosbie R. H., Utrophin to the rescue Nature 1996 384 6607 308 309
    • (1996) Nature , vol.384 , Issue.6607 , pp. 308-309
    • Campbell, K.P.1    Crosbie, R.H.2
  • 174
    • 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., Davies K. E., Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene Nature 1996 384 6607 349 353
    • (1996) Nature , vol.384 , Issue.6607 , 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
  • 175
    • 50549098032 scopus 로고    scopus 로고
    • Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin/Utrophin-deficient mice
    • Odom G. L., Gregorevic P., Allen J. M., Finn E., Chamberlain J. S., Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin/Utrophin-deficient mice Molecular Therapy 2008 16 9 1539 1545
    • (2008) Molecular Therapy , vol.16 , Issue.9 , pp. 1539-1545
    • Odom, G.L.1    Gregorevic, P.2    Allen, J.M.3    Finn, E.4    Chamberlain, J.S.5
  • 176
    • 0033427671 scopus 로고    scopus 로고
    • Nitric oxide and L-arginine cause an accumulation of utrophin at the sarcolemma: A possible compensation for dystrophin loss in Duchenne muscular dystrophy
    • Chaubourt E., Fossier P., Baux G., Leprince C., Israel M., De La Porte S., Nitric oxide and L-arginine cause an accumulation of utrophin at the sarcolemma: a possible compensation for dystrophin loss in Duchenne muscular dystrophy Neurobiology of Disease 1999 6 6 499 507
    • (1999) Neurobiology of Disease , vol.6 , Issue.6 , pp. 499-507
    • Chaubourt, E.1    Fossier, P.2    Baux, G.3    Leprince, C.4    Israel, M.5    De La Porte, S.6
  • 177
    • 28544447100 scopus 로고    scopus 로고
    • Systemic administration of L-arginine benefits mdx skeletal muscle function
    • Barton E. R., Morris L., Kawana M., Bish L. T., Toursel T., Systemic administration of L-arginine benefits mdx skeletal muscle function Muscle and Nerve 2005 32 6 751 760
    • (2005) Muscle and Nerve , vol.32 , Issue.6 , pp. 751-760
    • Barton, E.R.1    Morris, L.2    Kawana, M.3    Bish, L.T.4    Toursel, T.5
  • 178
    • 24044503385 scopus 로고    scopus 로고
    • L-arginine improves dystrophic phenotype in mdx mice
    • Voisin V., Sebrie C., Matecki S., L-arginine improves dystrophic phenotype in mdx mice Neurobiology of Disease 2005 20 1 123 130
    • (2005) Neurobiology of Disease , vol.20 , Issue.1 , pp. 123-130
    • Voisin, V.1    Sebrie, C.2    Matecki, S.3
  • 179
    • 72849123745 scopus 로고    scopus 로고
    • Mass spectrometry imaging of rat brain sections: Nanomolar sensitivity with MALDI versus nanometer resolution by TOF-SIMS
    • Benabdellah F., Seyer A., Quinton L., Touboul D., Brunelle A., Laprvote O., Mass spectrometry imaging of rat brain sections: nanomolar sensitivity with MALDI versus nanometer resolution by TOF-SIMS Analytical and Bioanalytical Chemistry 2010 396 1 151 162
    • (2010) Analytical and Bioanalytical Chemistry , vol.396 , Issue.1 , pp. 151-162
    • Benabdellah, F.1    Seyer, A.2    Quinton, L.3    Touboul, D.4    Brunelle, A.5    Laprvote, O.6
  • 180
    • 44849127317 scopus 로고    scopus 로고
    • L-arginine decreases inflammation and modulates the nuclear factor- B/matrix metalloproteinase cascade in Mdx muscle fibers
    • Hnia K., Gayraud J., Hugon G., L-arginine decreases inflammation and modulates the nuclear factor- B/matrix metalloproteinase cascade in Mdx muscle fibers American Journal of Pathology 2008 172 6 1509 1519
    • (2008) American Journal of Pathology , vol.172 , Issue.6 , pp. 1509-1519
    • Hnia, K.1    Gayraud, J.2    Hugon, G.3
  • 181
    • 13844303635 scopus 로고    scopus 로고
    • 7B integrin changes in mdx mouse muscles after L-arginine administration
    • Chazalette D., Hnia K., Rivier F., Hugon G., Mornet D., 7B integrin changes in mdx mouse muscles after L-arginine administration FEBS Letters 2005 579 5 1079 1084
    • (2005) FEBS Letters , vol.579 , Issue.5 , pp. 1079-1084
    • Chazalette, D.1    Hnia, K.2    Rivier, F.3    Hugon, G.4    Mornet, D.5
  • 182
    • 65249157858 scopus 로고    scopus 로고
    • Nitric oxide generated by muscle corrects defects in hippocampal neurogenesis and neural differentiation caused by muscular dystrophy
    • Deng B., Glanzman D., Tidball J. G., Nitric oxide generated by muscle corrects defects in hippocampal neurogenesis and neural differentiation caused by muscular dystrophy Journal of Physiology 2009 587 8 1769 1778
    • (2009) Journal of Physiology , vol.587 , Issue.8 , pp. 1769-1778
    • Deng, B.1    Glanzman, D.2    Tidball, J.G.3
  • 183
    • 0032978374 scopus 로고    scopus 로고
    • Activation of utrophin promoter by heregulin via the ets-related transcription factor complex GA-binding protein
    • Khurana T. S., Rosmarin A. G., Shang J., Krag T. O. B., Das S., Gammeltoft S., Activation of utrophin promoter by heregulin via the ets-related transcription factor complex GA-binding protein / Molecular Biology of the Cell 1999 10 6 2075 2086
    • (1999) Molecular Biology of the Cell , vol.10 , Issue.6 , pp. 2075-2086
    • Khurana, T.S.1    Rosmarin, A.G.2    Shang, J.3    Krag, T.O.B.4    Das, S.5    Gammeltoft, S.6
  • 185
    • 1042289645 scopus 로고    scopus 로고
    • Neuregulin-1- 1 enters brain and spinal cord by receptor-mediated transport
    • Kastin A. J., Akerstrom V., Pan W., Neuregulin-1- 1 enters brain and spinal cord by receptor-mediated transport Journal of Neurochemistry 2004 88 4 965 970
    • (2004) Journal of Neurochemistry , vol.88 , Issue.4 , pp. 965-970
    • Kastin, A.J.1    Akerstrom, V.2    Pan, W.3
  • 187
    • 33644975603 scopus 로고    scopus 로고
    • Utrophin upregulation for treating Duchenne or Becker muscular dystrophy: How close are we?
    • Miura P., Jasmin B. J., Utrophin upregulation for treating Duchenne or Becker muscular dystrophy: how close are we? Trends in Molecular Medicine 2006 12 3 122 129
    • (2006) Trends in Molecular Medicine , vol.12 , Issue.3 , pp. 122-129
    • Miura, P.1    Jasmin, B.J.2
  • 188
    • 10044237735 scopus 로고    scopus 로고
    • Glucocorticoid treatment alleviates dystrophic myofiber pathology by activation of the calcineurin/NF-AT pathway
    • St-Pierre S. J. G., Chakkalakal J. V., Kolodziejczyk S. M., Knudson J. C., Jasmin B. J., Megeney L. A., Glucocorticoid treatment alleviates dystrophic myofiber pathology by activation of the calcineurin/NF-AT pathway FASEB Journal 2004 18 15 1937 1939
    • (2004) FASEB Journal , vol.18 , Issue.15 , pp. 1937-1939
    • St-Pierre, S.J.G.1    Chakkalakal, J.V.2    Kolodziejczyk, S.M.3    Knudson, J.C.4    Jasmin, B.J.5    Megeney, L.A.6
  • 189
    • 0033200108 scopus 로고    scopus 로고
    • Expression of the utrophin gene during myogenic differentiation
    • Gramolini A. O., Jasmin B. J., Expression of the utrophin gene during myogenic differentiation Nucleic Acids Research 1999 27 17 3603 3609
    • (1999) Nucleic Acids Research , vol.27 , Issue.17 , pp. 3603-3609
    • Gramolini, A.O.1    Jasmin, B.J.2
  • 190
    • 70449388874 scopus 로고    scopus 로고
    • Pharmacological activation of PPAR / stimulates utrophin A expression in skeletal muscle fibers and restores sarcolemmal integrity in mature mdx mice
    • Miura P., Chakkalakal J. V., Boudreault L., Pharmacological activation of PPAR / stimulates utrophin A expression in skeletal muscle fibers and restores sarcolemmal integrity in mature mdx mice Human Molecular Genetics 2009 18 23 4640 4649
    • (2009) Human Molecular Genetics , vol.18 , Issue.23 , pp. 4640-4649
    • Miura, P.1    Chakkalakal, J.V.2    Boudreault, L.3
  • 192
    • 0037408464 scopus 로고    scopus 로고
    • Gentamicin fails to increase dystrophin expression in dystrophin-deficient muscle
    • Dunant P., Walter M. C., Karpati G., Lochmuller H., Gentamicin fails to increase dystrophin expression in dystrophin-deficient muscle Muscle and Nerve 2003 27 5 624 627
    • (2003) Muscle and Nerve , vol.27 , Issue.5 , pp. 624-627
    • Dunant, P.1    Walter, M.C.2    Karpati, G.3    Lochmuller, H.4
  • 193
    • 53749088724 scopus 로고    scopus 로고
    • Gentamicin treatment in exercised mdx mice: Identification of dystrophin-sensitive pathways and evaluation of efficacy in work-loaded dystrophic muscle
    • De Luca A., Nico B., Rolland J.-F., Gentamicin treatment in exercised mdx mice: identification of dystrophin-sensitive pathways and evaluation of efficacy in work-loaded dystrophic muscle Neurobiology of Disease 2008 32 2 243 253
    • (2008) Neurobiology of Disease , vol.32 , Issue.2 , pp. 243-253
    • De Luca, A.1    Nico, B.2    Rolland, J.-F.3
  • 194
    • 0034982292 scopus 로고    scopus 로고
    • Gentamicin treatment of Duchenne and Becker muscular dystrophy due to nonsense mutations
    • Wagner K. R., Hamed S., Hadley D. W., Gentamicin treatment of Duchenne and Becker muscular dystrophy due to nonsense mutations Annals of Neurology 2001 49 6 706 711
    • (2001) Annals of Neurology , vol.49 , Issue.6 , pp. 706-711
    • Wagner, K.R.1    Hamed, S.2    Hadley, D.W.3
  • 195
    • 70350448984 scopus 로고    scopus 로고
    • Nonaminoglycoside compounds induce readthrough of nonsense mutations
    • Du L., Damoiseaux R., Nahas S., Nonaminoglycoside compounds induce readthrough of nonsense mutations Journal of Experimental Medicine 2009 206 10 2285 2297
    • (2009) Journal of Experimental Medicine , vol.206 , Issue.10 , pp. 2285-2297
    • Du, L.1    Damoiseaux, R.2    Nahas, S.3
  • 196
    • 34247588271 scopus 로고    scopus 로고
    • PTC124 targets genetic disorders caused by nonsense mutations
    • Welch E. M., Barton E. R., Zhuo J., PTC124 targets genetic disorders caused by nonsense mutations Nature 2007 447 7140 87 91
    • (2007) Nature , vol.447 , Issue.7140 , pp. 87-91
    • Welch, E.M.1    Barton, E.R.2    Zhuo, J.3
  • 197
    • 33947529670 scopus 로고    scopus 로고
    • Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers
    • Hirawat S., Welch E. M., Elfring G. L., Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers Journal of Clinical Pharmacology 2007 47 4 430 444
    • (2007) Journal of Clinical Pharmacology , vol.47 , Issue.4 , pp. 430-444
    • Hirawat, S.1    Welch, E.M.2    Elfring, G.L.3
  • 198
    • 61649097962 scopus 로고    scopus 로고
    • Theoretic applicability of antisense-mediated exon skipping for Duchenne muscular dystrophy mutations
    • Aartsma-Rus A., Fokkema I., Verschuuren J., Theoretic applicability of antisense-mediated exon skipping for Duchenne muscular dystrophy mutations Human Mutation 2009 30 3 293 299
    • (2009) Human Mutation , vol.30 , Issue.3 , pp. 293-299
    • Aartsma-Rus, A.1    Fokkema, I.2    Verschuuren, J.3
  • 200
    • 69949107887 scopus 로고    scopus 로고
    • Local restoration of dystrophin expression with the morpholino oligomer AVI-4658 in Duchenne muscular dystrophy: A single-blind, placebo-controlled, dose-escalation, proof-of-concept study
    • Kinali M., Arechavala-Gomeza V., Feng L., Local restoration of dystrophin expression with the morpholino oligomer AVI-4658 in Duchenne muscular dystrophy: a single-blind, placebo-controlled, dose-escalation, proof-of-concept study The Lancet Neurology 2009 8 10 918 928
    • (2009) The Lancet Neurology , vol.8 , Issue.10 , pp. 918-928
    • Kinali, M.1    Arechavala-Gomeza, V.2    Feng, L.3
  • 202
    • 10044240371 scopus 로고    scopus 로고
    • Rescue of dystrophic muscle through U7 snRNA-mediated exon skipping
    • Goyenvalle A., Vulin A., Fougerousse F., Rescue of dystrophic muscle through U7 snRNA-mediated exon skipping Science 2004 306 5702 1796 1799
    • (2004) Science , vol.306 , Issue.5702 , pp. 1796-1799
    • Goyenvalle, A.1    Vulin, A.2    Fougerousse, F.3
  • 203
    • 67649849595 scopus 로고    scopus 로고
    • Enhanced exon-skipping induced by U7 snRNA carrying a splicing silencer sequence: Promising tool for DMD therapy
    • Goyenvalle A., Babbs A., van Ommen G.-J. B., Garcia L., Davies K. E., Enhanced exon-skipping induced by U7 snRNA carrying a splicing silencer sequence: promising tool for DMD therapy Molecular Therapy 2009 17 7 1234 1240
    • (2009) Molecular Therapy , vol.17 , Issue.7 , pp. 1234-1240
    • Goyenvalle, A.1    Babbs, A.2    Van Ommen, G.-J.B.3    Garcia, L.4    Davies, K.E.5
  • 204
    • 0042525989 scopus 로고    scopus 로고
    • Recombinant AAV serotype 1 transduction efficiency and tropism in the murine brain
    • Wang C., Wang C.-M., Clark K. R., Sferra T. J., Recombinant AAV serotype 1 transduction efficiency and tropism in the murine brain Gene Therapy 2003 10 17 1528 1534
    • (2003) Gene Therapy , vol.10 , Issue.17 , pp. 1528-1534
    • Wang, C.1    Wang, C.-M.2    Clark, K.R.3    Sferra, T.J.4
  • 205
    • 4344566457 scopus 로고    scopus 로고
    • Recombinant AAV viral vectors pseudotyped with viral capsids from serotypes 1, 2, and 5 display differential efficiency and cell tropism after delivery to different regions of the central nervous system
    • Burger C., Gorbatyuk O. S., Velardo M. J., Recombinant AAV viral vectors pseudotyped with viral capsids from serotypes 1, 2, and 5 display differential efficiency and cell tropism after delivery to different regions of the central nervous system Molecular Therapy 2004 10 2 302 317
    • (2004) Molecular Therapy , vol.10 , Issue.2 , pp. 302-317
    • Burger, C.1    Gorbatyuk, O.S.2    Velardo, M.J.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.