Outputs (20)

Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse
Journal Article
Miller, G., Moore, C. J., Terry, R. L., La Riviere, T., Mitchell, A., Piggott, R., Dear, T. N., Wells, D. J., & Winder, S. J. (in press). Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse. Human Molecular Genetics, 21(20), 4508-4520. https://doi.org/10.1093/hmg/dds293

Loss of dystrophin protein due to mutations in the DMD gene causes Duchenne muscular dystrophy. Dystrophin loss also leads to the loss of the dystrophin glycoprotein complex (DGC) from the sarcolemma which contributes to the dystrophic phenotype. Tyr... Read More about Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse.

MUSCLE PHYSIOLOGY PROPERTIES OF MOUSE MODELS FOR DUCHENNE MUSCULAR DYSTROPHY
Presentation / Conference Contribution
van Putten, M., Terry, R., Hulsker, M., Wells, K. E., Aartsma-Rus, A., & Wells, D. J. MUSCLE PHYSIOLOGY PROPERTIES OF MOUSE MODELS FOR DUCHENNE MUSCULAR DYSTROPHY

PEPTIDE-PMO INDUCED EXON-SKIPPING RESTORES MUSCLE PHYSIOLOGY IN THE MDX MOUSE
Presentation / Conference Contribution
Wells, K. E., Muses, S., Terry, R. L., Wood, M. J., Gait, M. J., & Wells, D. J. PEPTIDE-PMO INDUCED EXON-SKIPPING RESTORES MUSCLE PHYSIOLOGY IN THE MDX MOUSE

MUSCLE PHYSIOLOGY PROPERTIES OF MOUSE MODELS FOR DUCHENNE MUSCULAR DYSTROPHY
Presentation / Conference Contribution
van Putten, M., Terry, R. L., Hulsker, M., Wells, K. E., Aartsma-Rus, A., & Wells, D. J. MUSCLE PHYSIOLOGY PROPERTIES OF MOUSE MODELS FOR DUCHENNE MUSCULAR DYSTROPHY

Identification and validation of quantitative PCR reference genes suitable for normalising expression in normal and dystrophic cell culture models of myogenesis
Journal Article
Hildyard, J. C. W., & Wells, D. J. Identification and validation of quantitative PCR reference genes suitable for normalising expression in normal and dystrophic cell culture models of myogenesis. https://doi.org/10.1371/currents.md.faafdde4bea8df4aa7d06cd5553119a6

THE PHYSIOLOGICAL CONSEQUENCES OF DIFFERENT LEVELS OF DYSTROPHIN FOLLOWING ANTISENSE BASED EXON-SKIPPING IN THE MDX MOUSE
Presentation / Conference Contribution
Muses, S., Godfrey, C., McClorey, G., Wells, K. E., Coursindel, T., Terry, R. L., Betts, C., Cappellari, O., Hammond, S., O'Donovan, E., Hildyard, J. C. W., El Andaloussi, S., Gait, M., Wood, M., & Wells, D. J. THE PHYSIOLOGICAL CONSEQUENCES OF DIFFERENT LEVELS OF DYSTROPHIN FOLLOWING ANTISENSE BASED EXON-SKIPPING IN THE MDX MOUSE

We examined the effects on muscle physiology of restoring different levels of dystrophin in mdx mice with established dystrophic pathophysiology (12 weeks and older). Dystrophin expression was induced very efficiently using cell penetrating peptides... Read More about THE PHYSIOLOGICAL CONSEQUENCES OF DIFFERENT LEVELS OF DYSTROPHIN FOLLOWING ANTISENSE BASED EXON-SKIPPING IN THE MDX MOUSE.

Histopathological Evaluation of Skeletal Muscle with Specific Reference to Mouse Models of Muscular Dystrophy
Journal Article
Terry, R. L., & Wells, D. J. Histopathological Evaluation of Skeletal Muscle with Specific Reference to Mouse Models of Muscular Dystrophy. https://doi.org/10.1002/cpmo.19

Characterisation of the pathogenic effects of the in vivo expression of an ALS-linked mutation in D-amino acid oxidase: Phenotype and loss of spinal cord motor neurons
Journal Article
Kondori, N. R., Paul, P., Robbins, J. P., Liu, K., Hildyard, J. C. W., Wells, D. J., & De Belleroche, J. S. (in press). Characterisation of the pathogenic effects of the in vivo expression of an ALS-linked mutation in D-amino acid oxidase: Phenotype and loss of spinal cord motor neurons. PLoS ONE, 12, e0188912. https://doi.org/10.1371/journal.pone.0188912

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset neuromuscular disorder characterised by selective loss of motor neurons leading to fatal paralysis. Current therapeutic approaches are limited in their effectiveness. Substantial adva... Read More about Characterisation of the pathogenic effects of the in vivo expression of an ALS-linked mutation in D-amino acid oxidase: Phenotype and loss of spinal cord motor neurons.

CHARACTERISING THE SKELETAL MUSCLE HISTOLOGICAL PHENOTYPE OF THE DELTAE50-MD DOG, A PRECLINICAL MODEL OF DUCHENNE MUSCULAR DYSTROPHY
Presentation / Conference Contribution
Hildyard, J. C. W., Rawson, F., Harron, R., Riddell, D., Massey, C. A., Taylor-Brown, F. E., Wells, D. J., & Piercy, R. J. CHARACTERISING THE SKELETAL MUSCLE HISTOLOGICAL PHENOTYPE OF THE DELTAE50-MD DOG, A PRECLINICAL MODEL OF DUCHENNE MUSCULAR DYSTROPHY

Determination of qPCR Reference Genes Suitable for Normalizing Gene Expression in a Canine Model of Duchenne Muscular Dystrophy
Journal Article
Hildyard, J. C. W., Taylor-Brown, F. E., Massey, C., Wells, D. J., & Piercy, R. J. (in press). Determination of qPCR Reference Genes Suitable for Normalizing Gene Expression in a Canine Model of Duchenne Muscular Dystrophy. Journal of Neuromuscular Diseases, 5(2), 177-191. https://doi.org/10.3233/JND-170267

Background:Dogs with dystrophin-deficient muscular dystrophy are valuable models of the equivalent human disease, Duchenne Muscular Dystrophy (DMD): unlike the mdx mouse, these animals present a disease severity and progression that closely matches t... Read More about Determination of qPCR Reference Genes Suitable for Normalizing Gene Expression in a Canine Model of Duchenne Muscular Dystrophy.