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Identification of qPCR reference genes suitable for normalizing gene expression in the mdx mouse model of Duchenne muscular dystrophy (2019)
Journal Article
Hildyard, J. C. W., Finch, A. M., & Wells, D. J. (2019). Identification of qPCR reference genes suitable for normalizing gene expression in the mdx mouse model of Duchenne muscular dystrophy. PLoS ONE, 14(1), e0211384. https://doi.org/10.1371/journal.pone.0211384

The mdx mouse is the most widely-used animal model of the human disease Duchenne muscular dystrophy, and quantitative PCR analysis of gene expression in the muscles of this animal plays a key role in the study of pathogenesis and disease progression... Read More about Identification of qPCR reference genes suitable for normalizing gene expression in the mdx mouse model of Duchenne muscular dystrophy.

Focus on the Role of D-serine and D-amino Acid Oxidase in Amyotrophic Lateral Sclerosis/Motor Neuron Disease (ALS) (2018)
Journal Article
Kondori, N. R., Paul, P., Robbins, J. P., Liu, K., Hildyard, J. C. W., Wells, D. J., & De Belleroche, J. S. (2018). Focus on the Role of D-serine and D-amino Acid Oxidase in Amyotrophic Lateral Sclerosis/Motor Neuron Disease (ALS). Frontiers in Molecular Biosciences, 5(8), https://doi.org/10.3389/fmolb.2018.00008

Highly pathogenic avian influenza (HPAI) H5N1 virus has been circulating in Vietnam since 2003, while outbreaks of HPAI H5N6 virus are more recent, having only been reported since 2014. Although the spatial distribution of H5N1 outbreaks and risk fac... Read More about Focus on the Role of D-serine and D-amino Acid Oxidase in Amyotrophic Lateral Sclerosis/Motor Neuron Disease (ALS).

Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window? (2016)
Journal Article
Hildyard, J. C. W., Lacey, E., Booler, H., Hopkinson, M., Wells, D. J., & Brown, S. C. (2016). Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window?. PLoS ONE, 11(7), e0159853. https://doi.org/10.1371/journal.pone.0159853

LARGE is a glycosyltransferase involved in glycosylation of α-dystroglycan (α-DG). Absence of this protein in the LARGEmyd mouse results in α-DG hypoglycosylation, and is associated with central nervous system abnormalities and progressive muscular d... Read More about Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window?.

Poloxomer 188 Has a Deleterious Effect on Dystrophic Skeletal Muscle Function (2014)
Journal Article
Terry, R. L., Kaneb, H. M., & Wells, D. J. (2014). Poloxomer 188 Has a Deleterious Effect on Dystrophic Skeletal Muscle Function. PLoS ONE, 9(3), e91221. https://doi.org/10.1371/journal.pone.0091221

Duchenne muscular dystrophy (DMD) is an X-linked, fatal muscle wasting disease for which there is currently no cure and limited palliative treatments. Poloxomer 188 (P188) is a tri-block copolymer that has been proposed as a potential treatment for c... Read More about Poloxomer 188 Has a Deleterious Effect on Dystrophic Skeletal Muscle Function.

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., …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.

THE PHYSIOLOGICAL CONSEQUENCES OF DIFFERENT LEVELS OF DYSTROPHIN FOLLOWING ANTISENSE BASED EXON-SKIPPING IN THE MDX MOUSE
Journal Article
Muses, S., Godfrey, C., McClorey, G., Wells, K. E., Coursindel, T., Terry, R. L., …Wells, D. J. (in press). THE PHYSIOLOGICAL CONSEQUENCES OF DIFFERENT LEVELS OF DYSTROPHIN FOLLOWING ANTISENSE BASED EXON-SKIPPING IN THE MDX MOUSE. Neuromuscular Disorders, 25, S312-S313. https://doi.org/10.1016/j.nmd.2015.06.450

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.

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.

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.