GENERATION OF THREE-DIMENSIONAL HUMAN ARTIFICIAL SKELETAL MUSCLE TISSUE FROM IPS CELLS ENABLES COMPLEX DISEASE MODELLING FOR MUSCULAR DYSTROPHY
Presentation / Conference Contribution
Maffioletti, S. M., Sarcar, S., Henderson, A., Mannhardt, I., Pinton, L., Moyle, L. A., Steele-Stallard, H., Cappellari, O., Wells, K. E., Ragazzi, M., Wang, W., Zammit, P., Wells, D. J., Eschenhagen, T., & Tedesco, F. S. GENERATION OF THREE-DIMENSIONAL HUMAN ARTIFICIAL SKELETAL MUSCLE TISSUE FROM IPS CELLS ENABLES COMPLEX DISEASE MODELLING FOR MUSCULAR DYSTROPHY
Outputs (63)
Designing translationally relevant preclinical studies of new therapeutics
Journal Article
Wells, D. J. Designing translationally relevant preclinical studies of new therapeutics. Experimental Physiology, 102(6), 616. https://doi.org/10.1113/EP086296
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-170267Background: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., Wells, D. J., & Piercy, R. J. CHARACTERISING THE SKELETAL MUSCLE HISTOLOGICAL PHENOTYPE OF THE DELTAE50-MD DOG, A PRECLINICAL MODEL OF DUCHENNE MUSCULAR DYSTROPHY
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.0188912Amyotrophic 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.
Muscle moment arms and sensitivity analysis of a mouse hindlimb musculoskeletal model
Journal Article
Charles, J. P., Cappellari, O., Spence, A. J., Wells, D. J., & Hutchinson, J. R. (in press). Muscle moment arms and sensitivity analysis of a mouse hindlimb musculoskeletal model. Journal of Anatomy, 229(4), 514-535. https://doi.org/10.1111/joa.12461
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
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 MOUSEWe 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.
Implications for Cardiac Function Following Rescue of the Dystrophic Diaphragm in a Mouse Model of Duchenne Muscular Dystrophy
Journal Article
Betts, C. A., Saleh, A. F., Carr, C. A., Muses, S., Wells, K. E., Hammond, S. M., Godfrey, C., McClorey, G., Woffindale, C., Clarke, K., Wells, D. J. G. M. J., & Wood, M. J. A. (in press). Implications for Cardiac Function Following Rescue of the Dystrophic Diaphragm in a Mouse Model of Duchenne Muscular Dystrophy. https://doi.org/10.1038/srep11632
The TREAT-NMD advisory committee for therapeutics (TACT): an innovative de-risking model to foster orphan drug development
Journal Article
Heslop, E., Csimma, C., Straub, V., McCall, J., Nagaraju, K., Wagner, K. R., Caizergues, D., Korinthenberg, R., Flanigan, K. M., Kaufmann, P., McNeil, E., Mendell, J., Hesterlee, S., & Wells, D. J. (in press). The TREAT-NMD advisory committee for therapeutics (TACT): an innovative de-risking model to foster orphan drug development. Orphanet Journal of Rare Diseases, 10(49), https://doi.org/10.1186/s13023-015-0258-1
In silico characterisation and chromosomal localisation of human RRH (peropsin) – implications for opsin evolution
Journal Article
Bellingham, J., Wells, D. J., & Foster, R. G. In silico characterisation and chromosomal localisation of human RRH (peropsin) – implications for opsin evolution. BMC Genomics, 4(1), 3. https://doi.org/10.1186/1471-2164-4-3
INVESTIGATING THE EFFECTS OF DAO TRANSGENES ON THE SOD1(G93A) MOUSE MODEL OF AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Presentation / Conference Contribution
Rahmani-Kondori, N., de Belleroche, J., & Wells, D. J. INVESTIGATING THE EFFECTS OF DAO TRANSGENES ON THE SOD1(G93A) MOUSE MODEL OF AMYOTROPHIC LATERAL SCLEROSIS (ALS)
IMPROVING TRANSLATIONAL STUDIES THROUGH BETTER ANIMAL EXPERIMENTS: LESSONS FROM THE HUMAN RARE DISEASE COMMUNITY
Presentation / Conference Contribution
Wells, D. J. IMPROVING TRANSLATIONAL STUDIES THROUGH BETTER ANIMAL EXPERIMENTS: LESSONS FROM THE HUMAN RARE DISEASE COMMUNITY
THE TREAT-NMD ADVISORY COMMITTEE FOR THERAPEUTICS (TACT): AN INNOVATIVE DE-RISKING MODEL TO FOSTER ORPHAN DRUG DEVELOPMENT
Presentation / Conference Contribution
Wells, D. J., Heslop, E., Csimma, C., Straub, V., McCall, J., Nagaraju, K., Wagner, K., Caizergues, D., Korinthenberg, R., Flanigan, K., Mendell, J., Kelly, M., Kaufmann, P., McNeil, E., Robertson, A., Johnston, L., & Bushby, K. THE TREAT-NMD ADVISORY COMMITTEE FOR THERAPEUTICS (TACT): AN INNOVATIVE DE-RISKING MODEL TO FOSTER ORPHAN DRUG DEVELOPMENT
Translational and Regulatory Challenges for Exon Skipping Therapies
Journal Article
Aartsma-Rus, A., Ferlini, A., Goemans, N., Pasmooij, A. M. G., Wells, D. J., Bushby, K., Vroom, E., & Balabanov, P. Translational and Regulatory Challenges for Exon Skipping Therapies. Human Gene Therapy, 25(10), 885-892. https://doi.org/10.1089/hum.2014.086
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
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
ASSESSMENT OF NEUROMUSCULAR JUNCTION ABNORMALITIES INDUCED BY ALPHA-DYSTROGLYCAN GLYCOSYLATION DEFECTS
Presentation / Conference Contribution
Fernandez-Fuente, M., Kim, J., Wells, D. J., & Brown, S. C. ASSESSMENT OF NEUROMUSCULAR JUNCTION ABNORMALITIES INDUCED BY ALPHA-DYSTROGLYCAN GLYCOSYLATION DEFECTS
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., Hulsker, M., Wells, K. E., Aartsma-Rus, A., & Wells, D. J. MUSCLE PHYSIOLOGY PROPERTIES OF MOUSE MODELS FOR DUCHENNE MUSCULAR DYSTROPHY