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All Outputs (15)

Serum inflammatory cytokines as disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy (2022)
Journal Article
Piercy, R., Riddell, D., Hildyard, J., Harron, R., & Wells, D. (2022). Serum inflammatory cytokines as disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy. Neuromuscular Disorders, https://doi.org/10.1242/dmm.049394

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. The DE50-MD dog is a canine model of DMD that closely mimi... Read More about Serum inflammatory cytokines as disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy.

Longitudinal assessment of blood-borne musculoskeletal disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy (2022)
Journal Article
Riddell, D. O., Hildyard, J. C. W., Harron, R. C. M., Wells, D. J., & Piercy, R. J. (in press). Longitudinal assessment of blood-borne musculoskeletal disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy. Wellcome Open Research, https://doi.org/10.12688/wellcomeopenres.17398.1

Background: Duchenne muscular dystrophy (DMD) is a fatal muscle wasting disease affecting approximately 1 in 6000 male births worldwide. Due to their phenotypic similarity to human patients, large animal models are invaluable tools for pre-clinical t... Read More about Longitudinal assessment of blood-borne musculoskeletal disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy.

Validation of DE50-MD dogs as a model for the cognitive and biochemical defects in the brain phenotype of Duchenne muscular dystrophy (2022)
Journal Article
Crawford, A., Hildyard, J., Rushing, S., Wells, D., Diez Leon, M., & Piercy, R. (in press). Validation of DE50-MD dogs as a model for the cognitive and biochemical defects in the brain phenotype of Duchenne muscular dystrophy. eLife, https://doi.org/10.1242/dmm.049291

Duchenne muscular dystrophy (DMD), a fatal musculoskeletal disorder, is associated with neurodevelopmental problems and cognitive impairment caused by dystrophin deficiency in the brain. Dog models of DMD represent key translational tools to study... Read More about Validation of DE50-MD dogs as a model for the cognitive and biochemical defects in the brain phenotype of Duchenne muscular dystrophy.

Single-transcript multiplex in situ hybridisation reveals unique patterns of dystrophin isoform expression in the developing mammalian embryo (2020)
Journal Article
Hildyard, J. C. W., Crawford, A. H., Rawson, F., Riddell, D. O., Harron, R. C. M., & Piercy, R. J. (2020). Single-transcript multiplex in situ hybridisation reveals unique patterns of dystrophin isoform expression in the developing mammalian embryo. Wellcome Open Research, 5, 76. https://doi.org/10.12688/wellcomeopenres.15762.2

Background: The dystrophin gene has multiple isoforms: full-length dystrophin (dp427) is principally known for its expression in skeletal and cardiac muscle, but is also expressed in the brain, and several internal promoters give rise to shorter, N-t... Read More about Single-transcript multiplex in situ hybridisation reveals unique patterns of dystrophin isoform expression in the developing mammalian embryo.

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.

Gene editing restores dystrophin expression in a canine model of Duchenne muscular dystrophy (2018)
Journal Article
Amoasii, L., Hildyard, J. C. W., Li, H., Sanchez-Ortiz, E., Mireault, A., Caballero, D., …Olson, E. N. (2018). Gene editing restores dystrophin expression in a canine model of Duchenne muscular dystrophy. Science, 362(6410), 86-91. https://doi.org/10.1126/science.aau1549

Mutations in the gene encoding dystrophin, a protein that maintains muscle integrity and function, cause Duchenne muscular dystrophy (DMD). The deltaE50-MD dog model of DMD harbors a mutation corresponding to a mutational “hotspot” in the human DMD g... Read More about Gene editing restores dystrophin expression in a canine 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?.

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.

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., …Piercy, R. J. CHARACTERISING THE SKELETAL MUSCLE HISTOLOGICAL PHENOTYPE OF THE DELTAE50-MD DOG, A PRECLINICAL MODEL OF DUCHENNE MUSCULAR DYSTROPHY. Neuromuscular Disorders, 28, S18. https://doi.org/10.1016/S0960-8966%2818%2930342-0

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.