Research Topics

Genomes and Genes

animal muscular dystrophy

Summary

Webpages

  1. muscular dystrophy, animal
    buchta.lib.bioinfo.pl/meid:58847
  2. muscular dystrophy, animal
    lib.bioinfo.pl/meid:58847

Publications

  1. Direct observation of failing fibers in muscles of dystrophic mice provides mechanistic insight into muscular dystrophy
    Dennis R Claflin
    Department of Surgery, University of Michigan, BSRB, Rm 2027, 109 Zina Pitcher Place, Ann Arbor, MI 48109 2200, USA
    Am J Physiol Cell Physiol 294:C651-8
  2. Muscle-bone interactions in dystrophin-deficient and myostatin-deficient mice
    Eric Montgomery
    Department of Cellular Biology and Anatomy, Medical College of Georgia, Augusta, Georgia 30912, USA
    Anat Rec A Discov Mol Cell Evol Biol 286:814-22
  3. Relocalization of neuronal nitric oxide synthase (nNOS) as a marker for complete restoration of the dystrophin associated protein complex in skeletal muscle
    Kim E Wells
    Department of Neuromuscular Diseases, Imperial College Faculty of Medicine, Charing Cross Hospital, St Dunstan's Road, W6 8RP, London, UK
    Neuromuscul Disord 13:21-31
  4. Restoration of all dystrophin protein interactions by functional domains in trans does not rescue dystrophy
    K L Gardner
    Department of Molecular and Cellular Biochemistry, College of Medicine, The Ohio State University, Columbus, OH 43210, USA
    Gene Ther 13:744-51
  5. Disodium cromoglycate protects dystrophin-deficient muscle fibers from leakiness
    Maria Julia Marques
    Departamento de Anatomia, Instituto de Biologia, Universidade Estadual de Campinas, Campinas, São Paulo 13083 970, Brazil
    Muscle Nerve 37:61-7
  6. Expression and localization of protein inhibitor of neuronal nitric oxide synthase in Duchenne muscular dystrophy
    Y Guo
    Critical Care Division, Royal Victoria Hospital, Room L3 05, 687 Pine Avenue West, Montréal, Québec H3A 1A1, Canada
    Muscle Nerve 24:1468-75
  7. Helper (CD4(+)) and cytotoxic (CD8(+)) T cells promote the pathology of dystrophin-deficient muscle
    M J Spencer
    Department of Pediatrics, University of California at Los Angeles, California 90095-1606, USA
    Clin Immunol 98:235-43
  8. Acetylcholine receptor organization at the dystrophic extraocular muscle neuromuscular junction
    Maria Julia Marques
    Departamento de Anatomia, Instituto de Biologia, Universidade Estadual de Campinas UNICAMP, Campinas, São Paulo 13083 970, Brazil
    Anat Rec (Hoboken) 290:846-54
  9. Passive mechanical properties of maturing extensor digitorum longus are not affected by lack of dystrophin
    Andrew V Wolff
    Department of Mechanical Engineering, Virginia Polytechnic Institute and State University, Blacksburg, Virginia, USA
    Muscle Nerve 34:304-12
  10. Systemic delivery of morpholino oligonucleotide restores dystrophin expression bodywide and improves dystrophic pathology
    Julia Alter
    Muscle Cell Biology, MRC Clinical Science Centre, Hammersmith Hospital, Du Cane Road, London, W12 0NN, UK
    Nat Med 12:175-7

Scientific Experts

Detail Information

Webpages2

  1. muscular dystrophy, animal
    buchta.lib.bioinfo.pl/meid:58847
  2. muscular dystrophy, animal
    lib.bioinfo.pl/meid:58847

Publications62

  1. Direct observation of failing fibers in muscles of dystrophic mice provides mechanistic insight into muscular dystrophy
    Dennis R Claflin
    Department of Surgery, University of Michigan, BSRB, Rm 2027, 109 Zina Pitcher Place, Ann Arbor, MI 48109 2200, USA
    Am J Physiol Cell Physiol 294:C651-8
    ....
  2. Muscle-bone interactions in dystrophin-deficient and myostatin-deficient mice
    Eric Montgomery
    Department of Cellular Biology and Anatomy, Medical College of Georgia, Augusta, Georgia 30912, USA
    Anat Rec A Discov Mol Cell Evol Biol 286:814-22
    ..Likewise, increased muscle mass produces a marked increase in the external dimensions of muscle attachment sites, even when increased muscle size is accompanied by extensive fibrosis and muscle weakness...
  3. Relocalization of neuronal nitric oxide synthase (nNOS) as a marker for complete restoration of the dystrophin associated protein complex in skeletal muscle
    Kim E Wells
    Department of Neuromuscular Diseases, Imperial College Faculty of Medicine, Charing Cross Hospital, St Dunstan's Road, W6 8RP, London, UK
    Neuromuscul Disord 13:21-31
    ....
  4. Restoration of all dystrophin protein interactions by functional domains in trans does not rescue dystrophy
    K L Gardner
    Department of Molecular and Cellular Biochemistry, College of Medicine, The Ohio State University, Columbus, OH 43210, USA
    Gene Ther 13:744-51
    ....
  5. Disodium cromoglycate protects dystrophin-deficient muscle fibers from leakiness
    Maria Julia Marques
    Departamento de Anatomia, Instituto de Biologia, Universidade Estadual de Campinas, Campinas, São Paulo 13083 970, Brazil
    Muscle Nerve 37:61-7
    ..This study supports the protective effects of cromolyn in dystrophic muscles and further indicates its action against muscle fiber leakiness in muscles that are differently affected by the lack of dystrophin...
  6. Expression and localization of protein inhibitor of neuronal nitric oxide synthase in Duchenne muscular dystrophy
    Y Guo
    Critical Care Division, Royal Victoria Hospital, Room L3 05, 687 Pine Avenue West, Montréal, Québec H3A 1A1, Canada
    Muscle Nerve 24:1468-75
    ..Moreover, our results indicate that PIN is not an integral component of the dystrophin complex inside skeletal muscle fibers...
  7. Helper (CD4(+)) and cytotoxic (CD8(+)) T cells promote the pathology of dystrophin-deficient muscle
    M J Spencer
    Department of Pediatrics, University of California at Los Angeles, California 90095-1606, USA
    Clin Immunol 98:235-43
    ..These results indicate that T cells promote the mdx pathology and suggest that immune-based therapies may provide benefit to DMD patients...
  8. Acetylcholine receptor organization at the dystrophic extraocular muscle neuromuscular junction
    Maria Julia Marques
    Departamento de Anatomia, Instituto de Biologia, Universidade Estadual de Campinas UNICAMP, Campinas, São Paulo 13083 970, Brazil
    Anat Rec (Hoboken) 290:846-54
    ..These results suggest that the lack of dystrophin per se does not influence the distribution of acetylcholine receptors at the neuromuscular junction of spared extraocular muscles...
  9. Passive mechanical properties of maturing extensor digitorum longus are not affected by lack of dystrophin
    Andrew V Wolff
    Department of Mechanical Engineering, Virginia Polytechnic Institute and State University, Blacksburg, Virginia, USA
    Muscle Nerve 34:304-12
    ..Determining this threshold may have important clinical implications for treatments of muscular dystrophy involving physical activity...
  10. Systemic delivery of morpholino oligonucleotide restores dystrophin expression bodywide and improves dystrophic pathology
    Julia Alter
    Muscle Cell Biology, MRC Clinical Science Centre, Hammersmith Hospital, Du Cane Road, London, W12 0NN, UK
    Nat Med 12:175-7
    ..Although the level of dystrophin expression achieved varies considerably between muscles, antisense therapy may provide a realistic hope for the treatment of a majority of individuals with DMD...
  11. Constitutive properties, not molecular adaptations, mediate extraocular muscle sparing in dystrophic mdx mice
    John D Porter
    Department of Ophthalmology, Case Western Reserve University and The Research Institute of University Hospitals of Cleveland, 11100 Euclid Ave, Cleveland, Ohio 44106 5068, USA
    FASEB J 17:893-5
    ....
  12. Induction of dystrophin expression by exon skipping in mdx mice following intramuscular injection of antisense oligonucleotides complexed with PEG-PEI copolymers
    Jason H Williams
    Department of Pharmacology and Physiology, Drexel University College of Medicine, Mailstop 488, NCB 8302, Philadelphia, PA 19102, USA
    Mol Ther 14:88-96
    ..We conclude that low Mw PEI2000(PEG550) copolymers function as high-capacity, nontoxic AO carriers suitable for in vivo transfection of skeletal muscle and are promising compounds for potential use in molecular therapy of DMD...
  13. iNOS expression in dystrophinopathies can be reduced by somatic gene transfer of dystrophin or utrophin
    J P Louboutin
    Department of Molecular and Cellular Engineering, University of Pennsylvania, Philadelphia 19104, USA
    Mol Med 7:355-64
    ..CONCLUSIONS: These results suggest that iNOS could play a role in the physiopathology of DMD and that the abnormal expression of iNOS could be corrected by gene therapy...
  14. Localized expression of specific P2X receptors in dystrophin-deficient DMD and mdx muscle
    Taiwen Jiang
    Molecular Medicine Group, Institute of Biomedical and Biomolecular Sciences, School of Pharmacy and Biomedical Sciences, St Michael's Building, White Swan Road, Portsmouth PO1 2DT, UK
    Neuromuscul Disord 15:225-36
    ..There is, thus, a burst of production of several P2X receptor subtypes in regenerating dystrophic muscle, which may have implications for drug targets for this muscle pathology...
  15. Spectrin-like repeats from dystrophin and alpha-actinin-2 are not functionally interchangeable
    Scott Q Harper
    Department of Neurology, University of Washington School of Medicine, HSB Room K243, Box 357720, Seattle, WA 98195 7720, USA
    Hum Mol Genet 11:1807-15
    ....
  16. Negamycin restores dystrophin expression in skeletal and cardiac muscles of mdx mice
    Masayuki Arakawa
    Department of Life Sciences, The University of Tokyo, 3-8-1 Komaba, Tokyo 153-8902
    J Biochem (Tokyo) 134:751-8
    ..We conclude that negamycin is a promising new therapeutic candidate for DMD and other genetic diseases caused by nonsense mutations...
  17. Regenerated mdx mouse skeletal muscle shows differential mRNA expression
    B S Tseng
    Division of Child Neurology, Department of Neurology, University of California at San Francisco, San Francisco, California 94143, USA
    J Appl Physiol 93:537-45
    ..These discrepancies could provide candidates for salvage pathways that maintain skeletal muscle integrity in the absence of a functional dystrophin protein in mdx skeletal muscle...
  18. Involvement of TRPC in the abnormal calcium influx observed in dystrophic (mdx) mouse skeletal muscle fibers
    Clarisse Vandebrouck
    Département de Physiologie, Université Catholique de Louvain (UCL 5540, Avenue Hippocrate 55, 1200 Brussels, Belgium
    J Cell Biol 158:1089-96
    ....
  19. Aquaporins in skeletal muscle: reassessment of the functional role of aquaporin-4
    Antonio Frigeri
    Department of General and Environmental Physiology and Centre of Excellence in Comparative Genomics CEGBA, University of Bari, Bari, Italy
    FASEB J 18:905-7
    ..These data imply an important role for aquaporins in skeletal muscle physiology as well as an involvement of AQP4 in the molecular alterations that occur in the muscle of DMD patients...
  20. Herpes simplex virus VP22 enhances adenovirus-mediated microdystrophin gene transfer to skeletal muscles in dystrophin-deficient (mdx) mice
    Fu Xiong
    Department of Neurology, First Affiliated Hospital, Sun Yat Sen University, Guangzhou 510080, People s Republic of China
    Hum Gene Ther 18:490-501
    ....
  21. Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue
    T Haenggi
    Institute of Pharmacology and Toxicology, University of Zurich, Winterthurerstrasse 190, 8057, Zürich, Switzerland
    Cell Mol Life Sci 63:1614-31
    ..Here, we focus on recent studies of the DGC in brain, blood-brain barrier and choroid plexus, retina, and kidney and discuss the role of dystrophin isoforms and utrophin for assembly of the complex in these tissues...
  22. Enhanced expression of the alpha 7 beta 1 integrin reduces muscular dystrophy and restores viability in dystrophic mice
    D J Burkin
    Department of Cell and Structural Biology, University of Illinois, Urbana, Illinois 61801, USA
    J Cell Biol 152:1207-18
    ..A video that contrasts kyphosis, gait, joint contractures, and mobility in mdx/utr(-/-) and alpha 7BX2-mdx/utr(-/-) mice can be accessed at http://www.jcb.org/cgi/content/full/152/6/1207...
  23. Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin/utrophin-deficient mice
    Guy L Odom
    Department of Neurology, Senator Paul D Wellstone Muscular Dystrophy Cooperative Research Center, University of Washington School of Medicine, Seattle, Washington, USA
    Mol Ther 16:1539-45
    ..This approach may hold promise as a treatment option for DMD because it avoids the potential immune responses that are associated with the delivery of exogenous dystrophin...
  24. IGF-II ameliorates the dystrophic phenotype and coordinately down-regulates programmed cell death
    J Smith
    School of Biosciences, University of Birmingham, Edgbaston, Birmingham B15 2TT, UK
    Cell Death Differ 7:1109-18
    ..Targeting PCD in dystrophic muscles reduces both PCD and the classical features of dystrophic pathology in the mdx mouse suggesting that IGF-II is a strong candidate for therapeutic intervention in the dystrophinopathies...
  25. N-Acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice
    Nicholas P Whitehead
    Bosch Institute, School of Medical Sciences, University of Sydney F13, Sydney, NSW 2006, Australia
    J Physiol 586:2003-14
    ..These results offer the prospect that antioxidants such as NAC could have therapeutic potential for DMD patients...
  26. Between channels and tears: aim at ROS to save the membrane of dystrophic fibres
    Carlo Reggiani
    Department of Anatomy and Physiology, University of Padova, Padova, Italy
    J Physiol 586:1779
  27. Dystrophin delivery in dystrophin-deficient DMDmdx skeletal muscle by isogenic muscle-derived stem cell transplantation
    Makoto Ikezawa
    Growth and Development Laboratory, Children's Hospital of Pittsburgh, 3460 Fifth Avenue, Pittsburgh, PA 15213, USA
    Hum Gene Ther 14:1535-46
    ....
  28. Restoration of human dystrophin following transplantation of exon-skipping-engineered DMD patient stem cells into dystrophic mice
    Rachid Benchaouir
    Stem Cell Laboratory, Department of Neurological Sciences, Fondazione IRCCS Ospedale Maggiore Policlinico, Centro Dino Ferrari, University of Milan, Via F Sforza 35, 20122 Milan, Italy
    Cell Stem Cell 1:646-57
    ..These data demonstrate that autologous engrafting of blood or muscle-derived CD133+ cells, previously genetically modified to reexpress a functional dystrophin, represents a promising approach for DMD...
  29. Full-length dystrophin gene transfer to the mdx mouse in utero
    D P Reay
    Department of Neurology, University of Pittsburgh, Pittsburgh, PA 15213, USA
    Gene Ther 15:531-6
    ....
  30. Functionalized PEG-PEI copolymers complexed to exon-skipping oligonucleotides improve dystrophin expression in mdx mice
    Shashank R Sirsi
    School of Biomedical Engineering, Drexel University, Philadelphia, PA 19104, USA
    Hum Gene Ther 19:795-806
    ..Our data indicate that TAT-modified PEG-PEI copolymers are effective carriers for delivery of ESOs to skeletal muscle and are promising compounds for the therapeutic treatment of DMD...
  31. Mdx mice inducibly expressing dystrophin provide insights into the potential of gene therapy for duchenne muscular dystrophy
    A Ahmad
    Department of Pediatrics, Division of Medical Genetics, Duke University Medical Center, Durham, NC 27710, USA
    Hum Mol Genet 9:2507-15
    ..This model should be useful in future studies concerning the potential of genetic therapy for DMD, as well as other muscle disorders...
  32. Gene delivery to dystrophic muscle
    Kim E Wells
    Department of Cellular and Molecular Neuroscience, Division of Neuroscience and Mental Health, Imperial College, London, United Kingdom
    Methods Mol Biol 423:421-31
    ..Although it is unlikely that the electrotransfer approach will be useful clinically, it is an important experimental tool, particularly in testing potential immune responses to gene transfer in the absence of vector proteins...
  33. Electroporation of corrective nucleic acids (CNA) in vivo to promote gene correction in dystrophic muscle
    Robert M I Kapsa
    Howard Florey Institute and St Vincent s Hospital, Parkville, Victoria, Australia
    Methods Mol Biol 423:405-19
    ....
  34. Functional amounts of dystrophin produced by skipping the mutated exon in the mdx dystrophic mouse
    Qi Long Lu
    Muscle Cell Biology, MRC Clinical Science Centre, Hammersmith Hospital, Du Cane Road, London, W12 0NN, UK
    Nat Med 9:1009-14
    ..Our data establishes the realistic practicality of an approach that is applicable, in principle, to a majority of cases of severe dystrophinopathy...
  35. Enhanced in vivo delivery of antisense oligonucleotides to restore dystrophin expression in adult mdx mouse muscle
    K E Wells
    Department of Neuromuscular Diseases, Imperial College London, Charing Cross Hospital, W6 8RP London, UK
    FEBS Lett 552:145-9
    ..Although expression was transient, many of the corrected fibres initially showed levels of dystrophin expression well above the 20% of endogenous previously shown to be necessary for phenotypic correction of the dystrophic phenotype...
  36. Intraperitoneal administration of phosphorothioate antisense oligodeoxynucleotide against splicing enhancer sequence induced exon skipping in dystrophin mRNA expressed in mdx skeletal muscle
    Yasuhiro Takeshima
    Department of Pediatrics, Graduate School of Medicine, Kobe University, 7 5 2 Kusunoki cho, Chuo Ku, Kobe 650 0017, Japan
    Brain Dev 27:488-93
    ..These results showed that the intraperitoneally administered oligodeoxynucleotide could be transfected to nucleus of mdx skeletal muscle without any carrier and was able to induce exon skipping in vivo...
  37. Full-length dystrophin cDNA transfer into skeletal muscle of adult mdx mice by electroporation
    Tatsufumi Murakami
    Division of Neurology, Department of Internal Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki City, Okayama 701 0192, Japan
    Muscle Nerve 27:237-41
    ..Our data indicate that electroporation in vivo could introduce large full-length dystrophin cDNA into skeletal muscle of adult mdx mice...
  38. Distal mdx muscle groups exhibiting up-regulation of utrophin and rescue of dystrophin-associated glycoproteins exemplify a protected phenotype in muscular dystrophy
    Paul Dowling
    Department of Pharmacology, Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Belfield, Ireland
    Naturwissenschaften 89:75-8
    ..Thus distal mdx muscle groups provide a cellular system that naturally avoids myofibre degeneration which might be useful in the search for naturally occurring compensatory mechanisms in inherited skeletal muscle diseases...
  39. Autologous transplantation of muscle precursor cells modified with a lentivirus for muscular dystrophy: human cells and primate models
    Simon P Quenneville
    Unité de Recherche en Génétique Humaine, Centre de Recherche du CHUL, CHUQ, Faculté de Médecine, Université Laval, Sainte Foy, Québec, Canada
    Mol Ther 15:431-8
    ..The modification led to correct exon skipping and to the expression of a quasi-dystrophin in vitro and in vivo. These results demonstrate the feasibility of lentiviral-based ex vivo gene therapy for DMD...
  40. Identification of a novel population of muscle stem cells in mice: potential for muscle regeneration
    Zhuqing Qu-Petersen
    Growth and Development Laboratory, Children's Hospital of Pittsburgh, Department of Orthopaedic Surgery, University of Pittsburgh, PA 15260, USA
    J Cell Biol 157:851-64
    ..Our results suggest that this novel population of muscle-derived stem cells will significantly improve muscle cell-mediated therapies...
  41. Increased expression of deltaCaMKII isoforms in skeletal muscle regeneration: Implications in dystrophic muscle disease
    S Thomas Abraham
    Department of Pharmaceutical Sciences, Campbell University School of Pharmacy, PO 1090, Buies Creek, NC 27529, USA
    J Cell Biochem 97:621-32
    ..These data support a fundamental role for deltaCaMKII in the regeneration process of muscle fibers in normal and mdx skeletal muscle and may have important implications in the reparative process following muscle death...
  42. Exogenous Dp71 is a dominant negative competitor of dystrophin in skeletal muscle
    Sigalit Leibovitz
    Department of Molecular Cell Biology, The Weizmann Institute of Science, 76100, Rehovot, Israel
    Neuromuscul Disord 12:836-44
    ..Since Dp71 lacks the actin binding domain, it cannot form the essential linkage between the dystrophin associated proteins complex and the cytoskeleton...
  43. A web-accessible complete transcriptome of normal human and DMD muscle
    Marina Bakay
    Research Center for Genetic Medicine, George Washington University School of Medicine, Children s National Medical Center, 111 Michigan Avenue NW, Washington, DC 20010, USA
    Neuromuscul Disord 12:S125-41
    ..org/pga). These data enumerate the full range of molecular changes associated downstream of dystrophin deficiency, and provide a web-accessible platform to study the specificity of transcriptional pathway alterations in muscle disease...
  44. Regenerating more than muscle in muscular dystrophy
    Ahlke Heydemann
    Circulation 110:3290-2
  45. A quantitative study of bioenergetics in skeletal muscle lacking utrophin and dystrophin
    M A Cole
    MRC Biochemical and Clinical Magnetic Resonance Unit, Department of Biochemistry, South Parks Road, Oxford OX1 3QU, UK
    Neuromuscul Disord 12:247-57
    ..The data indicate that the severe abnormalities which are present in the absence of utrophin and dystrophin leave basic muscle energetics intact and appear confined to processes involving the sarcolemma...
  46. Pharmacological control of cellular calcium handling in dystrophic skeletal muscle
    Urs T Ruegg
    Pharmacology Group, School of Pharmacy, University of Lausanne BEP, CH 1015 Lausanne, Switzerland
    Neuromuscul Disord 12:S155-61
    ..Other cellular signalling pathways (e.g. nitric oxide) might also be affected...
  47. Molecular and cellular contractile dysfunction of dystrophic muscle from young mice
    Dawn A Lowe
    Department of Biochemistry, Molecular Biology Biophysics, University of Minnesota, 420 Delaware Street SE, MMC 388, Minneapolis, Minnesota 55455, USA
    Muscle Nerve 34:92-100
    ..Elucidating the molecular mechanisms underlying muscle weakness at the onset of disease is important for designing treatment strategies...
  48. The sparing of extraocular muscle in dystrophinopathy is lost in mice lacking utrophin and dystrophin
    J D Porter
    Department of Ophthalmology, Case Western Reserve University, Cleveland, OH 44106 5068, USA
    J Cell Sci 111:1801-11
    ..Moreover, data lend support to the hypothesis that interventions designed to increase utrophin levels may ameliorate the pathology in other skeletal muscles in Duchenne muscular dystrophy...
  49. Branched fibers in dystrophic mdx muscle are associated with a loss of force following lengthening contractions
    S Chan
    School of Medical Sciences, Univ of New South Wales, Sydney 2052, Australia
    Am J Physiol Cell Physiol 293:C985-92
    ..Our findings demonstrate that fiber branching may play a role in the pathogenesis of muscular dystrophy in mdx mice, and this could affect the interpretation of previous studies involving lengthening contractions in this animal...
  50. rAAV6-microdystrophin rescues aberrant Golgi complex organization in mdx skeletal muscles
    Justin M Percival
    Department of Physiology and Biophysics, University of Washington, Box 357290, 1959 NE Pacific Street, Seattle, WA 98195, USA
    Traffic 8:1424-39
    ..In summary, GC distribution abnormalities are a novel component of mdx skeletal muscle pathology rescued by microdystrophin expression...
  51. Loss of sarcolemma nNOS in sarcoglycan-deficient muscle
    Rachelle H Crosbie
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    FASEB J 16:1786-91
    ..Our data suggest that loss of nNOS may contribute to muscle pathology in AR-LGMD with primary mutations in the sarcoglycans...
  52. Decreased myocardial nNOS, increased iNOS and abnormal ECGs in mouse models of Duchenne muscular dystrophy
    B L Bia
    Department of Biochemistry, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    J Mol Cell Cardiol 31:1857-62
    ..We conclude that lack of dystrophin in the mdx mouse results in abnormal ECGs that are associated with decreased myocardial nNOS and increased iNOS activities...
  53. Voltage-gated sodium channel (SkM1) content in dystrophin-deficient muscle
    P Ribaux
    Université Claude Bernard Lyon 1, Laboratoire de Physiologie des Eléments Excitables, Villeurbanne, France
    Pflugers Arch 441:746-55
    ..However, the moderate reduction in SkM1 density (-12.7%) observed in mdx muscle argues in favour of a non-exclusive role of syntrophins in SkM1 anchorage and suggests that other membrane-associated proteins are probably also involved...
  54. Contraction-induced injury to single permeabilized muscle fibers from mdx, transgenic mdx, and control mice
    G S Lynch
    Institute of Gerontology, University of Michigan, Ann Arbor, Michigan 48109 2007, USA
    Am J Physiol Cell Physiol 279:C1290-4
    ....
  55. Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle
    Sascha Wieneke
    Developmental Biology and Molecular Pathology, Bielefeld University, D 33501 Bielefeld, Germany
    J Appl Physiol 95:1861-6
    ..The adverse effects of Dp71 in muscle are probably due to its competition with dystrophin and utrophin (in MDX muscle) for binding to the DAP complex...
  56. Central tolerance to myogenic cell transplants does not include muscle neoantigens
    Geoffrey Camirand
    Internal Medicine, Yale University School of Medicine, New Haven, CT, USA
    Transplantation 85:1791-801
    ..This suggests that muscle-specific infiltration may result from lack of negative selection for peripheral neoantigens in the thymus after BMT and that tolerance after MT may rely on peripheral regulatory mechanisms...
  57. Regeneration-blocked mdx muscle: in vivo model for testing treatments
    J G Quinlan
    Department of Neurology, University of Cincinnati, Ohio 45237 0525, USA
    Muscle Nerve 20:1016-23
    ..Regeneration-blocked normal muscle showed stunted growth but neither progressive wasting nor microscopic pathological changes...
  58. Extracorporeal circulation as a new experimental pathway for myoblast implantation in mdx mice
    Y Torrente
    Centro Dino Ferrari, Institute of Clinical Neurology, University of Milan, Italy
    Cell Transplant 8:247-58
    ..2% of the total muscle fibers. These results demonstrate that the extracorporeal circulation allows selective myoblast-mediated gene transfer to muscles, opening new perspectives in muscular dystrophy gene therapy...
  59. Noninvasive monitoring of therapeutic gene transfer in animal models of muscular dystrophies
    M Bartoli
    Généthon, Centre National de la Recherche Scientifique UMR 8115, Evry, France
    Gene Ther 13:20-8
    ..The system described here provides a simple and noninvasive procedure for monitoring the outcome of a therapeutic strategy involving cell survival...
  60. Immune evasion by muscle-specific gene expression in dystrophic muscle
    C J Kirk
    Department of Neurology, University of Washington School of Medicine, HSB Room K243, Seattle, Washington 98195 7720, USA
    Mol Ther 4:525-33
    ..This result suggests that cross-presentation is not more effective in mdx mouse muscle, and that targeted vectors and tissue-specific promoters may be useful tools for evasion of the immune response in dystrophic muscle...