Results 1 to 10 of about 108 (65)

Validation of DE50-MD dogs as a model for the brain phenotype of Duchenne muscular dystrophy [PDF]

DMM Disease Models and Mechanisms, 2022
Duchenne muscular dystrophy (DMD), a fatal musculoskeletal disease, is associated with neurodevelopmental disorders and cognitive impairment caused by brain dystrophin deficiency.
Dominic Wells, Richard Piercy, Abbé Crawford   +2 more
exaly   +7 more sources

Longitudinal assessment of skeletal muscle functional mechanics in the DE50-MD dog model of Duchenne muscular dystrophy [PDF]

DMM Disease Models and Mechanisms, 2023
ABSTRACT Duchenne muscular dystrophy (DMD), caused by mutations in the dystrophin (DMD) gene, is associated with fatal muscle degeneration and atrophy. Patients with DMD have progressive reductions in skeletal muscle strength and resistance to eccentric muscle stretch.
Rachel Harron, Dominic Wells, Dominique Riddell   +2 more
exaly   +6 more sources

Serum inflammatory cytokines as disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy [PDF]

DMM Disease Models and Mechanisms, 2022
ABSTRACT Duchenne muscular dystrophy (DMD) is a fatal muscle-wasting disease, caused by mutations in the dystrophin gene, characterised by cycles of muscle degeneration, inflammation and regeneration. Recently, there has been renewed interest specifically in drugs that ameliorate muscle inflammation in DMD patients.
Rachel Harron, Dominic Wells, Dominique Riddell   +2 more
exaly   +6 more sources

Brain magnetic resonance imaging in the DE50-MD dog model of Duchenne muscular dystrophy reveals regional reductions in cerebral gray matter [PDF]

BMC Neuroscience, 2023
Background Duchenne muscular dystrophy is a X-linked disease characterized by severe and progressive muscle weakness, alongside cognitive impairment and a range of neurobehavioral disorders secondary to brain dystrophin deficiency.
Alerie Guzman De La Fuente, Richard Piercy, Abbé Crawford   +2 more
exaly   +9 more sources

Long-term, age-associated activity quantification in the DE50-MD dog model of Duchenne muscular dystrophy [PDF]

DMM Disease Models and Mechanisms
ABSTRACT Animal models with a clinically relevant phenotype remain important for robust evaluation of novel therapeutics for the fatal X-linked genetic disorder Duchenne muscular dystrophy (DMD). Demonstration of functional improvement is crucial for both patients and regulatory authorities.
Emil Olsen, Mónica A Daley, Richard Piercy   +2 more
exaly   +6 more sources

Longitudinal assessment of blood-borne musculoskeletal disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy [version 2; peer review: 2 approved] [PDF]

Wellcome Open Research, 2022
Background: Duchenne muscular dystrophy (DMD) is a fatal muscle wasting disease caused by mutations in the dystrophin gene. Due to their phenotypic similarity to human patients, large animal models are invaluable tools for pre-clinical trials.
John C. W. Hildyard, Dominic J. Wells, Rachel C. M. Harron, Dominique O. Riddell, Richard J. Piercy   +4 more
doaj   +10 more sources

The skeletal muscle phenotype of the DE50-MD dog model of Duchenne muscular dystrophy [version 1; peer review: 2 approved] [PDF]

Wellcome Open Research, 2022
Background: Animal models of Duchenne muscular dystrophy (DMD) are essential to study disease progression and assess efficacy of therapeutic intervention, however dystrophic mice fail to display a clinically relevant phenotype, limiting translational ...
Claire Massey, Emma M.A. Foster, Dominic J. Wells, Frances Taylor-Brown, Richard J. Piercy, Dominique O. Riddell, John C.W. Hildyard, Faye Rawson, Rachel C.M. Harron   +8 more
doaj   +7 more sources

Evaluation of a six-minute walk test in the DE50-MD canine model of Duchenne muscular dystrophy and its effect on blood-borne biomarkers [version 2; peer review: 1 approved, 2 approved with reservations] [PDF]

Wellcome Open Research
Background Duchenne muscular dystrophy (DMD) is a fatal muscle wasting disease caused by mutations in the dystrophin gene resulting in cycles of muscle degeneration, inflammation and regeneration. The 6-minute walk test (6MWT) is a key functional outcome
Richard Piercy, Rachel Harron, Dominique Riddell, Dominic Wells, John Hildyard   +4 more
doaj   +8 more sources

Musculoskeletal magnetic resonance imaging in the DE50-MD dog model of Duchenne muscular dystrophy [PDF]

Neuromuscular Disorders, 2021
The DE50-MD canine model of Duchenne muscular dystrophy (DMD) has a dystrophin gene splice site mutation causing deletion of exon 50, an out-of-frame transcript and absence of dystrophin expression in striated muscles. We hypothesized that the musculoskeletal phenotype of DE50-MD dogs could be detected using Magnetic Resonance Imaging (MRI), that it ...
Rachel Harron, Dominic Wells, Richard Piercy   +2 more
exaly   +5 more sources

Identification of quantitative polymerase chain reaction reference genes suitable for normalising gene expression in the brain of normal and dystrophic mice and dogs [version 2; peer review: 2 approved, 1 approved with reservations] [PDF]

Wellcome Open Research, 2023
Background: In addition to progressive, debilitating muscle degeneration, ~50% of patients with Duchenne muscular dystrophy (DMD) have associated cognitive and behavioural disorders secondary to deficiency of dystrophin protein in the brain.
Abbe H. Crawford, Dominic J. Wells, John C. W. Hildyard, Richard J. Piercy   +3 more
doaj   +2 more sources