Second-generation compound for the modulation of utrophin in the therapy of DMD. (Q41374222)

30 September 2017

Second-generation compound for the modulation of utrophin in the therapy of DMD. (English)

1 reference

30 September 2017

Benjamin Edwards

3

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Angela J Russell

object named as

Angela J Russell

10

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Kay Davies

14

object named as

Kay E Davies

1 reference

30 September 2017

6

Nandini Shah

1 reference

30 September 2017

7

Arran Babbs

1 reference

30 September 2017

Simon Guiraud

1

1 reference

30 September 2017

Sarah E Squire

2

1 reference

30 September 2017

Huijia Chen

4

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30 September 2017

David T Burns

5

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30 September 2017

Stephen G Davies

8

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30 September 2017

Graham M Wynne

9

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30 September 2017

David Elsey

11

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30 September 2017

Francis X Wilson

12

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30 September 2017

Jon M Tinsley

13

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30 September 2017

1 May 2015

1 reference

30 September 2017

Human Molecular Genetics

1 reference

30 September 2017

24

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30 September 2017

15

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30 September 2017

4212-4224

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describes a project that uses

30 September 2017

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4492389/fullTextXML

inferred from PubMed Central ID database lookup

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Arginine butyrate per os protects mdx mice against cardiomyopathy, kyphosis and changes in axonal excitability.

1 October 2017

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Muscle structure influences utrophin expression in mdx mice.

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PDE5 inhibition alleviates functional muscle ischemia in boys with Duchenne muscular dystrophy

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Phase 2a study of ataluren-mediated dystrophin production in patients with nonsense mutation Duchenne muscular dystrophy

1 October 2017

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1 October 2017

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AMP-activated protein kinase at the nexus of therapeutic skeletal muscle plasticity in Duchenne muscular dystrophy

1 October 2017

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A cell-based high-throughput screening assay for posttranscriptional utrophin upregulation

1 October 2017

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Nitric oxide signalling pathway in Duchenne muscular dystrophy mice: up-regulation of L-arginine transporters

1 October 2017

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1 October 2017

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Successful regional delivery and long-term expression of a dystrophin gene in canine muscular dystrophy: a preclinical model for human therapies

1 October 2017

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Increased resting intracellular calcium modulates NF-κB-dependent inducible nitric-oxide synthase gene expression in dystrophic mdx skeletal myotubes

1 October 2017

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Daily treatment with SMTC1100, a novel small molecule utrophin upregulator, dramatically reduces the dystrophic symptoms in the mdx mouse

1 October 2017

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Discovery of 2-arylbenzoxazoles as upregulators of utrophin production for the treatment of Duchenne muscular dystrophy.

1 October 2017

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Biglycan recruits utrophin to the sarcolemma and counters dystrophic pathology in mdx mice

1 October 2017

1 reference

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Sarcolemmal nNOS anchoring reveals a qualitative difference between dystrophin and utrophin.

1 October 2017

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Calcium influx is sufficient to induce muscular dystrophy through a TRPC-dependent mechanism

1 October 2017

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Early manifestation of alteration in cardiac function in dystrophin deficient mdx mouse using 3D CMR tagging

1 October 2017

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Functional substitution by TAT-utrophin in dystrophin-deficient mice

1 October 2017

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Modulation of utrophin A mRNA stability in fast versus slow muscles via an AU-rich element and calcineurin signaling.

1 October 2017

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Bex1 knock out mice show altered skeletal muscle regeneration

1 October 2017

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Molecular mechanisms of muscular dystrophies: old and new players

1 October 2017

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Utrophin upregulation for treating Duchenne or Becker muscular dystrophy: how close are we?

1 October 2017

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Heregulin ameliorates the dystrophic phenotype in mdx mice

1 October 2017

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Therapeutics for Duchenne muscular dystrophy: current approaches and future directions.

1 October 2017

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Cardiomyopathy in muscular dystrophies

1 October 2017

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Expression of utrophin A mRNA correlates with the oxidative capacity of skeletal muscle fiber types and is regulated by calcineurin/NFAT signaling

1 October 2017

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Assumption-free analysis of quantitative real-time polymerase chain reaction (PCR) data

1 October 2017

1 reference

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Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method

1 October 2017

1 reference

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A new mathematical model for relative quantification in real-time RT-PCR

1 October 2017

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Molecular basis of muscular dystrophies.

1 October 2017

1 reference

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Different dystrophin-like complexes are expressed in neurons and glia

1 October 2017

1 reference

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

1 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

1 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

1 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Contractile properties of skeletal muscles from young, adult and aged mice

1 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

An autosomal transcript in skeletal muscle with homology to dystrophin

1 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

1 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Studying the role of dystrophin-associated proteins in influencing Becker muscular dystrophy disease severity.

1 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Flexibility in the N-terminal actin-binding domain: clues from in silico mutations and molecular dynamics.

11 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Pathological pattern of Mdx mice diaphragm correlates with gradual expression of the short utrophin isoform Up71.

11 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

11 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Human dystrophin gene transfer: production and expression of a functional recombinant DNA-based gene

11 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Utrophin A is essential in mediating the functional adaptations of mdx mouse muscle following chronic AMPK activation.

11 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Safety and efficacy of drisapersen for the treatment of Duchenne muscular dystrophy (DEMAND II): an exploratory, randomised, placebo-controlled phase 2 study

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Utrophin regulates modal gating of mechanosensitive ion channels in dystrophic skeletal muscle.

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Ex vivo gene editing of the dystrophin gene in muscle stem cells mediated by peptide nucleic acid single stranded oligodeoxynucleotides induces stable expression of dystrophin in a mouse model for Duchenne muscular dystrophy

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

A comparison of factors associated with collagen metabolism in different skeletal muscles from dystrophic (mdx) mice: impact of pirfenidone.

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Central role for the cardiotonic steroid marinobufagenin in the pathogenesis of experimental uremic cardiomyopathy

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Localization and early time course of TGF-beta 1 mRNA expression in dystrophic muscle

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Regenerative capacity of the dystrophic (mdx) diaphragm after induced injury

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Drastic reduction of calsequestrin-like proteins and impaired calcium binding in dystrophic mdx muscle

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Dystroglycan mRNA expression during normal and mdx mouse embryogenesis: a comparison with utrophin and the apo-dystrophins.

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Duchenne muscular dystrophy--Meryon's disease.

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Expression of dystrophin-associated glycoproteins during human fetal muscle development: a preliminary immunocytochemical study.

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Dystrophin, dystrophin-associated protein and dystrophinopathy

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Efficient utrophin expression following adenovirus gene transfer in dystrophic muscle

19 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

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

4 November 2018

1 reference

Crossref

https://api.crossref.org/works/10.1093%2FHMG%2FDDV154

Severe muscle dysfunction precedes collagen tissue proliferation in mdx mouse diaphragm

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4492389

Voluntary exercise induces structural remodeling in the hearts of dystrophin-deficient mice

2 December 2018

1 reference

12 December 2020

Diaphragm rescue alone prevents heart dysfunction in dystrophic mice

1 reference

12 December 2020

Power output of fast and slow skeletal muscles of mdx (dystrophic) and control mice after clenbuterol treatment

1 reference

12 December 2020

Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophy

1 reference

12 December 2020

Effects of UCP3 genotype, temperature and muscle type on energy turnover of resting mouse skeletal muscle

1 reference

12 December 2020

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

1 reference

12 December 2020

Evolution of the mdx mouse cardiomyopathy: physiological and morphological findings

1 reference

12 December 2020

Management of end stage respiratory failure in Duchenne muscular dystrophy

1 reference

12 December 2020

Identification of FHL1 as a therapeutic target for Duchenne muscular dystrophy

1 reference

12 December 2020

1 reference

12 December 2020

Differential expression of muscular dystrophy in diaphragm versus hindlimb muscles of mdx mice

1 reference

12 December 2020

The dystrophin-related protein, utrophin, is expressed on the sarcolemma of regenerating human skeletal muscle fibres in dystrophies and inflammatory myopathies

1 reference

12 December 2020

1 reference

12 December 2020

Expression of the dystrophin-related protein (utrophin) gene during mouse embryogenesis

1 reference

12 December 2020

Morphometric analysis of mdx diaphragm muscle fibres. Comparison with hindlimb muscles

1 reference

12 December 2020

Identifiers

DOI

10.1093/HMG/DDV154

1 reference

30 September 2017

1 reference

30 September 2017

PubMed publication ID

25935002

1 reference