Duchenne's muscular dystrophy: animal models used to investigate pathogenesis and develop therapeutic strategies

scientific article published on August 2003

Duchenne's muscular dystrophy: animal models used to investigate pathogenesis and develop therapeutic strategies is …
instance of (P31):
scholarly articleQ13442814
review articleQ7318358

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P356DOI10.1046/J.1365-2613.2003.00354.X
P932PMC publication ID2517561
P698PubMed publication ID14632630
P5875ResearchGate publication ID8994827

P2093author name stringJ E Morgan
C A Collins
P2860cites workX chromosome-linked muscular dystrophy (mdx) in the mouseQ28589078
Normal myoblast injections provide genetic treatment for murine dystrophyQ68420325
Muscle precursor cells invade and repopulate freeze-killed musclesQ68781971
Mdx muscle grafts retain the mdx phenotype in normal hostsQ69617523
Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouseQ70039598
Muscle development in mdx mutant miceQ70804155
Low-dose prednisolone treatment in Duchenne and Becker muscular dystrophyQ71952881
Expression of utrophin (dystrophin-related protein) during regeneration and maturation of skeletal muscle in canine X-linked muscular dystrophyQ72377687
[Muscular dystrophy in a cat]Q72628350
Canine X-linked muscular dystrophy in Japan (CXMDJ)Q73525127
Myoblast transplantation in whole muscle of nonhuman primatesQ73614007
In vivo targeted repair of a point mutation in the canine dystrophin gene by a chimeric RNA/DNA oligonucleotideQ73844338
Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of miceQ73851362
T-cell-dependent fibrosis in the mdx dystrophic mouseQ73952718
Development of muscle pathology in canine X-linked muscular dystrophy. II. Quantitative characterization of histopathological progression during postnatal skeletal muscle developmentQ74310479
Dystrophin in adult zebrafish muscleQ74393273
Changes of skeletal muscle in young dystrophin-deficient cats: a morphological and morphometric studyQ74408052
The worm in us - Caenorhabditis elegans as a model of human diseaseQ77800192
Tibialis anterior muscles in mdx mice are highly susceptible to contraction-induced injuryQ77974446
Cardiomyopathy in dystrophin-deficient hypertrophic feline muscular dystrophyQ78130933
Dystrophin: the protein product of the Duchenne muscular dystrophy locusQ29618077
Molecular analysis of a spontaneous dystrophin 'knockout' dog.Q30735735
The dystrophin associated protein complex in zebrafishQ31134324
Utrophin binds laterally along actin filaments and can couple costameric actin with sarcolemma when overexpressed in dystrophin-deficient muscleQ33787827
Progress in myoblast transplantation: a potential treatment of dystrophiesQ33840950
Muscular dystrophy: the worm turns to genetic diseaseQ34085566
Harnessing the potential of dystrophin-related proteins for ameliorating Duchenne's muscular dystrophyQ34285869
Long-term persistence and migration of myogenic cells injected into pre-irradiated muscles of mdx miceQ34306103
Murine muscular dystrophy caused by a mutation in the laminin alpha 2 (Lama2) geneQ34319775
Gene transfer studies in animals: what do they really tell us about the prospects for gene therapy in DMD?Q34807072
Viral vectors for gene transfer of micro-, mini-, or full-length dystrophinQ34807084
Oligonucleotide-mediated gene therapy for muscular dystrophiesQ34807121
Dystrophin-glycoprotein complex: post-translational processing and dystroglycan function.Q35056189
Non-viral gene delivery in skeletal muscle: a protein factoryQ35061706
Zebrafish: from disease modeling to drug discovery.Q35096379
Current protocol of a research phase I clinical trial of full-length dystrophin plasmid DNA in Duchenne/Becker muscular dystrophies. Part I: rationale.Q40635064
Normal myogenic cells from newborn mice restore normal histology to degenerating muscles of the mdx mouseQ41902045
Deficiency of merosin in dystrophic dy mice and genetic linkage of laminin M chain gene to dy locusQ42501151
Conversion of mdx myofibres from dystrophin-negative to -positive by injection of normal myoblastsQ43465378
Comparative evolution of muscular dystrophy in diaphragm, gastrocnemius and masseter muscles from old male mdx miceQ43717688
Muscle lesions associated with dystrophin deficiency in neonatal golden retriever puppiesQ43955218
Efficacy of myoblast transplantation in nonhuman primates following simple intramuscular cell injections: toward defining strategies applicable to humansQ43992517
Remission of clinical signs in early duchenne muscular dystrophy on intermittent low-dosage prednisolone therapyQ44167443
Prevention of pathology in mdx mice by expression of utrophin: analysis using an inducible transgenic expression system.Q44241921
Enhanced dystrophic progression in mdx mice by exercise and beneficial effects of taurine and insulin-like growth factor-1.Q44255035
Dystrophic phenotype of canine X-linked muscular dystrophy is mitigated by adenovirus-mediated utrophin gene transfer.Q44409495
Use of the dog model for Duchenne muscular dystrophy in gene therapy trials.Q44434265
Tarsal joint contracture in dogs with golden retriever muscular dystrophyQ44864009
High-efficiency plasmid gene transfer into dystrophic muscleQ45859128
Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigeneQ45865580
Stable micro-dystrophin gene transfer using an integrating adeno-retroviral hybrid vector ameliorates the dystrophic pathology in mdx mouse muscle.Q45887463
Myoblast implantation in Duchenne muscular dystrophy: the San Francisco study.Q45956233
In vitro interactions of Caenorhabditis elegans dystrophin with dystrobrevin and syntrophin.Q47068721
Mutations in the Caenorhabditis elegans dystrophin-like gene dys-1 lead to hyperactivity and suggest a link with cholinergic transmissionQ47068751
Dystrobrevin- and dystrophin-like mutants display similar phenotypes in the nematode Caenorhabditis elegans.Q47068987
Restoration of dystrophin expression in mdx muscle cells by chimeraplast-mediated exon skippingQ47947457
The dystrophin / utrophin homologues in Drosophila and in sea urchinQ48378746
Canine X-linked muscular dystrophy. An animal model of Duchenne muscular dystrophy: clinical studies.Q50892927
High-level dystrophin expression after adenovirus-mediated dystrophin minigene transfer to skeletal muscle of dystrophic dogs: prolongation of expression with immunosuppression.Q53956299
A New Immunodeficient Mouse Model for Human Myoblast TransplantationQ57268530
The homologue of the Duchenne locus is defective in X-linked muscular dystrophy of dogsQ59051983
Fibrogenic cytokines and extent of fibrosis in muscle of dogs with X-linked golden retriever muscular dystrophyQ61828055
The mdx mouse skeletal muscle myopathy: II. Contractile propertiesQ68025103
Ultrastructure of the skeletal muscle in the X chromosome-linked dystrophic (mdx) mouse. Comparison with Duchenne muscular dystrophyQ68055667
P433issue4
P921main subjectmuscular dystrophyQ1137767
P304page(s)165-172
P577publication date2003-08-01
P1433published inInternational Journal of Experimental PathologyQ15750807
P1476titleDuchenne's muscular dystrophy: animal models used to investigate pathogenesis and develop therapeutic strategies
P478volume84

Reverse relations

cites work (P2860)
Q35761079A COLQ Missense Mutation in Sphynx and Devon Rex Cats with Congenital Myasthenic Syndrome
Q45303122A Nematode Nobel Prize: Caenorhabditis elegans
Q33810272A mathematical model of skeletal muscle disease and immune response in the mdx mouse.
Q28576151Age-related dystrophin-glycoprotein complex structure and function in the rat extensor digitorum longus and soleus muscle
Q30497616An intronic LINE-1 element insertion in the dystrophin gene aborts dystrophin expression and results in Duchenne-like muscular dystrophy in the corgi breed
Q37059675Animal models for genetic neuromuscular diseases
Q34708380Bmi1 enhances skeletal muscle regeneration through MT1-mediated oxidative stress protection in a mouse model of dystrophinopathy
Q34665595Bortezomib (PS-341) treatment decreases inflammation and partially rescues the expression of the dystrophin-glycoprotein complex in GRMD dogs
Q36392294Challenges for gene therapy for muscular dystrophy
Q50422172Clinical and genetic characterisation of dystrophin-deficient muscular dystrophy in a family of Miniature Poodle dogs.
Q92996733Comparative proteomic analyses of Duchenne muscular dystrophy and Becker muscular dystrophy muscles: changes contributing to preserve muscle function in Becker muscular dystrophy patients
Q33290644Corticortophin releasing factor 2 receptor agonist treatment significantly slows disease progression in mdx mice
Q81820857Degeneration of dystrophic or injured skeletal muscles induces high expression of Galectin-1
Q30856794Development and use of DNA archives at veterinary teaching hospitals to investigate the genetic basis of disease in dogs
Q36501691Dilated cardiomyopathy: a tale of cytoskeletal proteins and beyond
Q24791491Diseased muscles that lack dystrophin or laminin-alpha2 have altered compositions and proliferation of mononuclear cell populations
Q38480698Drug Discovery of Therapies for Duchenne Muscular Dystrophy
Q47704855Duchenne and Becker Muscular Dystrophies: A Review of Animal Models, Clinical End Points, and Biomarker Quantification.
Q35911460Duchenne muscular dystrophy and dystrophin: pathogenesis and opportunities for treatment
Q35052506Duchenne muscular dystrophy gene therapy: Lost in translation?
Q83044679Eccentric stimulation reveals an involvement of FGF6 in muscle resistance to mechanical stress
Q90734610Effects of non-euphoric plant cannabinoids on muscle quality and performance of dystrophic mdx mice
Q33840407Excitation-contraction coupling alterations in mdx and utrophin/dystrophin double knockout mice: a comparative study
Q37271171Extracellular microRNAs are dynamic non-vesicular biomarkers of muscle turnover
Q64898517Extracellular microRNAs exhibit sequence-dependent stability and cellular release kinetics.
Q37396408Gene therapy in large animal models of muscular dystrophy
Q59128911Genetic and pharmacological regulation of the endocannabinoid CB1 receptor in Duchenne muscular dystrophy
Q36444141Genetic elevation of sphingosine 1-phosphate suppresses dystrophic muscle phenotypes in Drosophila
Q92922123Genome Editing for the Understanding and Treatment of Inherited Cardiomyopathies
Q37899626Getting priorities straight: risk assessment and decision-making in the improvement of inherited disorders in pedigree dogs
Q36832437Identification of disease specific pathways using in vivo SILAC proteomics in dystrophin deficient mdx mouse.
Q30571229In vitro Differentiation of Functional Human Skeletal Myotubes in a Defined System
Q41151043Increased plasma lipid levels exacerbate muscle pathology in the mdx mouse model of Duchenne muscular dystrophy
Q34691050Induction of revertant fibres in the mdx mouse using antisense oligonucleotides
Q41096343Influence of Botulinumtoxin A on the Expression of Adult MyHC Isoforms in the Masticatory Muscles in Dystrophin-Deficient Mice (Mdx-Mice).
Q36707428Inhibition of CaMKII phosphorylation of RyR2 prevents inducible ventricular arrhythmias in mice with Duchenne muscular dystrophy.
Q27438151Inhibitory control over Ca(2+) sparks via mechanosensitive channels is disrupted in dystrophin deficient muscle but restored by mini-dystrophin expression
Q46202556Intestine of dystrophic mice presents enhanced contractile resistance to stretching despite morphological impairment
Q36178999Invertebrate muscles: muscle specific genes and proteins
Q37184680Kinematics of gait in golden retriever muscular dystrophy.
Q33649312Long-term treatment with naproxcinod significantly improves skeletal and cardiac disease phenotype in the mdx mouse model of dystrophy
Q47074096Loss of selenoprotein N function causes disruption of muscle architecture in the zebrafish embryo.
Q26991681Model organisms in the fight against muscular dystrophy: lessons from drosophila and Zebrafish
Q36748510Modeling inflammatory bowel disease: the zebrafish as a way forward.
Q53180281Molecular hydrogen alleviates motor deficits and muscle degeneration in mdx mice.
Q35142741Motor physical therapy affects muscle collagen type I and decreases gait speed in dystrophin-deficient dogs.
Q40422606Muscle costameric protein, Chisel/Smpx, associates with focal adhesion complexes and modulates cell spreading in vitro via a Rac1/p38 pathway.
Q30578452Myogenic potential of canine craniofacial satellite cells.
Q40490992Non-Targeted Metabolomics Analysis of Golden Retriever Muscular Dystrophy-Affected Muscles Reveals Alterations in Arginine and Proline Metabolism, and Elevations in Glutamic and Oleic Acid In Vivo
Q28072269Nutraceuticals and Their Potential to Treat Duchenne Muscular Dystrophy: Separating the Credible from the Conjecture
Q28547478Profiles of Steroid Hormones in Canine X-Linked Muscular Dystrophy via Stable Isotope Dilution LC-MS/MS
Q38391853Quantitative proteome profiling of dystrophic dog skeletal muscle reveals a stabilized muscular architecture and protection against oxidative stress after systemic delivery of MuStem cells.
Q59700731Retracted: ‘The effects of Duchenne muscular dystrophy on the performance of the stomatognathic system: case-control study’, by Ferreira B., Da Silva G.P., Gonçalves C.R., et al
Q39456770Semen analysis of Golden Retriever healthy dogs and those affected by muscular dystrophy.
Q46245053Skeletal Muscle Regenerative Potential of Human MuStem Cells following Transplantation into Injured Mice Muscle.
Q27304985Spatio-Temporal Differences in Dystrophin Dynamics at mRNA and Protein Levels Revealed by a Novel FlipTrap Line
Q50704888Stem cells from umbilical cord blood differentiate into myotubes and express dystrophin in vitro only after exposure to in vivo muscle environment.
Q36004069Stem cells isolated from human dental pulp and amniotic fluid improve skeletal muscle histopathology in mdx/SCID mice
Q53143745Stomatognathic function in Duchenne muscular dystrophy: a case-control study.
Q36841572Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy
Q55109195Utrophin haploinsufficiency does not worsen the functional performance, resistance to eccentric contractions and force production of dystrophic mice.
Q38351283What has the mdx mouse model of Duchenne muscular dystrophy contributed to our understanding of this disease?

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