Pharmacological strategies for muscular dystrophy (Q35128906)

6 August 2017

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title

Pharmacological strategies for muscular dystrophy (English)

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6 August 2017

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2

Kay E Davies

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6 August 2017

Tejvir S. Khurana

1

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A12750741

3 January 2024

Kay E. Davies

series ordinal

2

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A12750741

3 January 2024

language of work or name

English

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National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=12750741

3 January 2024

publication date

1 May 2003

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full work available at URL

6 August 2017

https://www.nature.com/articles/nrd1085.pdf

file format

Portable Document Format

1 reference

https://api.crossref.org/works/10.1038/NRD1085

https://www.nature.com/articles/nrd1085

3 January 2024

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https://api.crossref.org/works/10.1038/NRD1085

Nature Reviews Drug Discovery

3 January 2024

published in

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6 August 2017

volume

2

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6 August 2017

issue

5

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6 August 2017

page(s)

379-390

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Isolation of candidate cDNAs for portions of the Duchenne muscular dystrophy gene

6 August 2017

cites work

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Dystrophin: the protein product of the Duchenne muscular dystrophy locus

31 March 2020

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The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein

31 March 2020

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An autosomal transcript in skeletal muscle with homology to dystrophin

31 March 2020

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Primary structure of dystrophin-related protein

31 March 2020

1 reference

Characterization of DRP2, a novel human dystrophin homologue

31 March 2020

1 reference

(CA) repeat polymorphism in the chromosome 18 encoded dystrophin-like protein

31 March 2020

1 reference

Cloning and characterization of the human homologue of a dystrophin related phosphoprotein found at the Torpedo electric organ post-synaptic membrane

31 March 2020

1 reference

Isoform diversity of dystrobrevin, the murine 87-kDa postsynaptic protein

31 March 2020

1 reference

Three muscular dystrophies: loss of cytoskeleton-extracellular matrix linkage.

31 March 2020

1 reference

Membrane organization of the dystrophin-glycoprotein complex

31 March 2020

1 reference

Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle

31 March 2020

1 reference

Small-caliber skeletal muscle fibers do not suffer deleterious consequences of dystrophic gene expression

31 March 2020

1 reference

Extraocular muscles are spared in advanced Duchenne dystrophy

31 March 2020

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Absence of extraocular muscle pathology in Duchenne's muscular dystrophy: role for calcium homeostasis in extraocular muscle sparing

31 March 2020

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Animal models for muscular dystrophy: valuable tools for the development of therapies

31 March 2020

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X chromosome-linked muscular dystrophy (mdx) in the mouse

31 March 2020

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The homologue of the Duchenne locus is defective in X-linked muscular dystrophy of dogs

31 March 2020

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Molecular analysis of a spontaneous dystrophin 'knockout' dog.

31 March 2020

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Recovery of induced mutations for X chromosome-linked muscular dystrophy in mice

31 March 2020

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The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy

31 March 2020

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Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch

31 March 2020

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Dystrophin protects the sarcolemma from stresses developed during muscle contraction

31 March 2020

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The physiological evaluation of gene therapies of dystrophin-deficient muscles

31 March 2020

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Harnessing the potential of dystrophin-related proteins for ameliorating Duchenne's muscular dystrophy

31 March 2020

1 reference

Multivariate evaluation of the functional recovery obtained by the overexpression of utrophin in skeletal muscles of the mdx mouse

31 March 2020

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Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy

31 March 2020

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Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy

31 March 2020

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T-cell-dependent fibrosis in the mdx dystrophic mouse

31 March 2020

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The 2.0 A structure of the second calponin homology domain from the actin-binding region of the dystrophin homologue utrophin

31 March 2020

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Dystrophin and utrophin: the missing links!

31 March 2020

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Function and genetics of dystrophin and dystrophin-related proteins in muscle

31 March 2020

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Absence of alpha-syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin

31 March 2020

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Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse

31 March 2020

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Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle.

31 March 2020

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Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in prol

31 March 2020

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31 March 2020

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The subcellular distribution of dystrophin in mouse skeletal, cardiac, and smooth muscle

31 March 2020

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31 March 2020

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Dystrophin and dystrophin-related protein (utrophin) distribution in normal and dystrophin-deficient skeletal muscles

31 March 2020

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Differential expression of dystrophin, utrophin, and dystrophin-associated proteins in human muscle culture

31 March 2020

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Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice

31 March 2020

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Subtle neuromuscular defects in utrophin-deficient mice

31 March 2020

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Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice.

31 March 2020

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Expression of full-length utrophin prevents muscular dystrophy in mdx mice

31 March 2020

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Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene

31 March 2020

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Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene

31 March 2020

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The utrophin and dystrophin genes share similarities in genomic structure

31 March 2020

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A study of short utrophin isoforms in mice deficient for full-length utrophin

31 March 2020

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Molecular and functional analysis of the utrophin promoter

31 March 2020

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A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy

31 March 2020

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Utrophin transcription is activated by an intronic enhancer

31 March 2020

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A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle

31 March 2020

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Targeting transcription to the neuromuscular synapse

31 March 2020

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Neuregulin and ErbB receptor signaling pathways in the nervous system

31 March 2020

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Induction of utrophin gene expression by heregulin in skeletal muscle cells: role of the N-box motif and GA binding protein

31 March 2020

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Activation of utrophin promoter by heregulin via the ets-related transcription factor complex GA-binding protein alpha/beta

31 March 2020

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Sp1 and Sp3 physically interact and co-operate with GABP for the activation of the utrophin promoter

31 March 2020

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The role of basal and myogenic factors in the transcriptional activation of utrophin promoter A: implications for therapeutic up-regulation in Duchenne muscular dystrophy

31 March 2020

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Sp1 and the ets-related transcription factor complex GABP alpha/beta functionally cooperate to activate the utrophin promoter.

31 March 2020

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Ets, Ap-1 and GATA factor families regulate the utrophin B promoter: potential regulatory mechanisms for endothelial-specific expression

31 March 2020

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Transcriptional activation of the utrophin promoter B by a constitutively active Ets-transcription factor

31 March 2020

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Nitric oxide and l-arginine cause an accumulation of utrophin at the sarcolemma: a possible compensation for dystrophin loss in Duchenne muscular dystrophy

31 March 2020

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Muscular nitric oxide synthase (muNOS) and utrophin

31 March 2020

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Enhanced expression of the alpha 7 beta 1 integrin reduces muscular dystrophy and restores viability in dystrophic mice

31 March 2020

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Overexpression of the cytotoxic T cell GalNAc transferase in skeletal muscle inhibits muscular dystrophy in mdx mice

31 March 2020

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Increased expression of utrophin in a slow vs. a fast muscle involves posttranscriptional events

31 March 2020

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Glucocorticoid-mediated regulation of utrophin levels in human muscle fibers

31 March 2020

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Non-toxic ubiquitous over-expression of utrophin in the mdx mouse

31 March 2020

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

31 March 2020

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Dystrophic phenotype of canine X-linked muscular dystrophy is mitigated by adenovirus-mediated utrophin gene transfer.

31 March 2020

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Current protocol of a research phase I clinical trial of full-length dystrophin plasmid DNA in Duchenne/Becker muscular dystrophies. Part I: rationale.

31 March 2020

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Current protocol of a research phase I clinical trial of full-length dystrophin plasmid DNA in Duchenne/Becker muscular dystrophies. Part II: clinical protocol

31 March 2020

1 reference

Prednisone in Duchenne muscular dystrophy

31 March 2020

1 reference

Randomized, double-blind six-month trial of prednisone in Duchenne's muscular dystrophy

31 March 2020

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Steroids in Duchenne muscular dystrophy; pilot study of a new low-dosage schedule

31 March 2020

1 reference

A multicenter, double-blind, randomized trial of deflazacort versus prednisone in Duchenne muscular dystrophy

31 March 2020

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Intracellular calcium accumulation in Duchenne dystrophy and other myopathies: a study of 567,000 muscle fibers in 114 biopsies

31 March 2020

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Expression profiling reveals metabolic and structural components of extraocular muscles

31 March 2020

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Increased activity of calcium leak channels in myotubes of Duchenne human and mdx mouse origin

31 March 2020

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Proteolysis results in altered leak channel kinetics and elevated free calcium in mdx muscle.

31 March 2020

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Long-term study of Ca(2+) homeostasis and of survival in collagenase-isolated muscle fibres from normal and mdx mice

31 March 2020

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Effect of dantrolene in Duchenne muscular dystrophy

31 March 2020

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Delay of muscle degeneration and necrosis in mdx mice by calpain inhibition.

31 March 2020

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Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology

31 March 2020

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Mitochondrial calcium overload: A general mechanism for cell-necrosis in muscle diseases

31 March 2020

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Monoclonal antibody analysis of mononuclear cells in myopathies. I: Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells

31 March 2020

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Monoclonal antibody analysis of mononuclear cells in myopathies. II: Phenotypes of autoinvasive cells in polymyositis and inclusion body myositis

31 March 2020

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Monoclonal antibody analysis of mononuclear cells in myopathies. III: Immunoelectron microscopy aspects of cell-mediated muscle fiber injury

31 March 2020

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A role for mast cells in the progression of Duchenne muscular dystrophy? Correlations in dystrophin-deficient humans, dogs, and mice

31 March 2020

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Expression profiling in the muscular dystrophies: identification of novel aspects of molecular pathophysiology

31 March 2020

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Gene expression comparison of biopsies from Duchenne muscular dystrophy (DMD) and normal skeletal muscle

31 March 2020

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Elevated basic fibroblast growth factor in the serum of patients with Duchenne muscular dystrophy

31 March 2020

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Helper (CD4(+)) and cytotoxic (CD8(+)) T cells promote the pathology of dystrophin-deficient muscle

31 March 2020

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Duchenne-like myopathy in double-mutant mdx mice expressing exaggerated mast cell activity

31 March 2020

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Clenbuterol, a beta 2-agonist, retards wasting and loss of contractility in irradiated dystrophic mdx muscle

31 March 2020

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Creatine supplementation improves intracellular Ca2+ handling and survival in mdx skeletal muscle cells

31 March 2020

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Creatine monohydrate in muscular dystrophies: A double-blind, placebo-controlled clinical study

31 March 2020

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Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice

31 March 2020

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Muscle-specific expression of insulin-like growth factor I counters muscle decline in mdx mice

31 March 2020

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Improved contractile function of the mdx dystrophic mouse diaphragm muscle after insulin-like growth factor-I administration

31 March 2020

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Enhanced dystrophic progression in mdx mice by exercise and beneficial effects of taurine and insulin-like growth factor-1.

31 March 2020

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The mh gene causing double-muscling in cattle maps to bovine Chromosome 2.

31 March 2020

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A deletion in the bovine myostatin gene causes the double-muscled phenotype in cattle

31 March 2020

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Regulation of skeletal muscle mass in mice by a new TGF-beta superfamily member

31 March 2020

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A missense mutant myostatin causes hyperplasia without hypertrophy in the mouse muscle

31 March 2020

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Dominant negative myostatin produces hypertrophy without hyperplasia in muscle

31 March 2020

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GDF-8 propeptide binds to GDF-8 and antagonizes biological activity by inhibiting GDF-8 receptor binding

31 March 2020

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Regulation of myostatin activity and muscle growth

31 March 2020

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Myostatin, a negative regulator of muscle growth, functions by inhibiting myoblast proliferation

31 March 2020

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The myostatin gene is a downstream target gene of basic helix-loop-helix transcription factor MyoD.

31 March 2020

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Myostatin inhibits myoblast differentiation by down-regulating MyoD expression.

31 March 2020

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Functional improvement of dystrophic muscle by myostatin blockade.

31 March 2020

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Loss of myostatin attenuates severity of muscular dystrophy in mdx mice

31 March 2020

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Collaborative translational research leading to multicenter clinical trials in Duchenne muscular dystrophy: the Cooperative International Neuromuscular Research Group (CINRG).

31 March 2020

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Pre-clinical screening of drugs using the mdx mouse

31 March 2020

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Efficient adenovirus-mediated transfer of a human minidystrophin gene to skeletal muscle of mdx mice

31 March 2020

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Antisense-induced exon skipping restores dystrophin expression in DMD patient derived muscle cells

31 March 2020

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In vivo targeted repair of a point mutation in the canine dystrophin gene by a chimeric RNA/DNA oligonucleotide

31 March 2020

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Rescue of dystrophin expression in mdx mouse muscle by RNA/DNA oligonucleotides

31 March 2020

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Conversion of mdx myofibres from dystrophin-negative to -positive by injection of normal myoblasts

31 March 2020

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Recruitment of bone-marrow-derived cells by skeletal and cardiac muscle in adult dystrophic mdx mice

31 March 2020

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ERF: an ETS domain protein with strong transcriptional repressor activity, can suppress ets-associated tumorigenesis and is regulated by phosphorylation during cell cycle and mitogenic stimulation

31 March 2020

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Different distributions of dystrophin and related proteins at nerve-muscle junctions

31 March 2020

series ordinal

45

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https://api.crossref.org/works/10.1038/NRD1085

3 January 2024

Identifiers

DOI

10.1038/NRD1085

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Dimensions Publication ID

6 August 2017

1009739627

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