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All Outputs (93)

Musculoskeletal Geometry, Muscle Architecture and Functional Specialisations of the Mouse Hindlimb (2016)
Journal Article
Charles, J. P., Cappellari, O., Spence, A. J., Hutchinson, J. R., & Wells, D. J. (2016). Musculoskeletal Geometry, Muscle Architecture and Functional Specialisations of the Mouse Hindlimb. PLoS ONE, 11(4), https://doi.org/10.1371/journal.pone.0147669

Mice are one of the most commonly used laboratory animals, with an extensive array of disease models in existence, including for many neuromuscular diseases. The hindlimb is of particular interest due to several close muscle analogues/homologues to h... Read More about Musculoskeletal Geometry, Muscle Architecture and Functional Specialisations of the Mouse Hindlimb.

FHL1 activates myostatin signalling in skeletal muscle and promotes atrophy (2015)
Journal Article
Lee, J. Y., Lori, D., Wells, D. J., & Kemp, P. R. (2015). FHL1 activates myostatin signalling in skeletal muscle and promotes atrophy. FEBS Open Bio, 5, 753-762. https://doi.org/10.1016/j.fob.2015.08.011

Myostatin is a TGFβ family ligand that reduces muscle mass. In cancer cells, TGFβ signalling is increased by the protein FHL1. Consequently, FHL1 may promote signalling by myostatin. We therefore tested the ability of FHL1 to regulate myostatin funct... Read More about FHL1 activates myostatin signalling in skeletal muscle and promotes atrophy.

How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouse (2015)
Journal Article
Godfrey, C., Muses, S., McClorey, G., Wells, K. E., Coursindel, T., Terry, R. L., Betts, C., Hammond, S., O'Donovan, L., Hildyard, J. C. W., El Andaloussi, S., Gait, M. J., Wood, M. J., & Wells, D. J. (2015). How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouse. Human Molecular Genetics, 24(15), 4225-4237. https://doi.org/10.1093/hmg/ddv155

DEVELOPING, TESTING AND OPTIMISING A MOUSE HINDLIMB MUSCULOSKELETAL MODEL (2015)
Presentation / Conference Contribution
Charles, J. P., Cappellari, O., Spence, A., Wells, D. J., & Hutchinson, J. R. DEVELOPING, TESTING AND OPTIMISING A MOUSE HINDLIMB MUSCULOSKELETAL MODEL

Stability during locomotion arises from many complex interactions which are not yet fully understood. Studies into how sensory feedback from muscle spindles contributes to this have used neuromechanical simulations, however their accuracy is limited... Read More about DEVELOPING, TESTING AND OPTIMISING A MOUSE HINDLIMB MUSCULOSKELETAL MODEL.

Measuring Clinical Effectiveness of Medicinal Products for the Treatment of Duchenne Muscular Dystrophy (2015)
Journal Article
Lynn, S., Aartsma-Rus, A., Furlong, P., Goemans, N., De Luca, A., Mayhew, A., McDonald, C., Mercuri, E., Muntoni, F., Pohlschmidt, M., Verschuuren, J., Voit, T., Vroom, E., Wells, D. J., & Straub, V. (2015). Measuring Clinical Effectiveness of Medicinal Products for the Treatment of Duchenne Muscular Dystrophy. Neuromuscular Disorders, 25(1), 96-105. https://doi.org/10.1016/j.nmd.2014.09.003

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.

Local restoration of dystrophin expression with the morpholino oligomer AVI-4658 in Duchenne muscular dystrophy: a single-blind, placebo-controlled, dose-esclation, proof-of-concept study (2009)
Journal Article
Kinali, M., Arechavala-Gomeza, V., Feng, L., Cirak, S., Hunt, D., Adkin, C., Guglieri, M., Ashton, E., Abbs, S., Nihoyannopoulos, P., Garralda, M. E., Rutherford, M., McCulley, C., Popplewell, L., Graham, I. R., Dickson, G., Wood, M. J., Wells, D. J., Wilton, S. D., Kole, R., …Muntoni, F. (2009). Local restoration of dystrophin expression with the morpholino oligomer AVI-4658 in Duchenne muscular dystrophy: a single-blind, placebo-controlled, dose-esclation, proof-of-concept study. https://doi.org/10.1016/S1474-4422%2809%2970211-X

EXON SKIPPING AND DYSTROPHIN RESTORATION IN DUCHENNE MUSCULAR DYSTROPHY PATIENTS AFTER SYSTEMIC PHOSPHORODIAMIDATE MORPHOLINO OLIGOMER TREATMENT
Presentation / Conference Contribution
Cirak, S., Arechavala-Gomeza, V., Guglieri, M., Feng, L., Torelli, S., Anthony, K., Garralda, M. E., Wells, D. J., Dickson, G., Wood, M. J. A., Wilton, S. D., Straub, V., Shrewsbury, S. B., Sewry, C., Morgan, J. E., Bushby, K., & Muntoni, F. EXON SKIPPING AND DYSTROPHIN RESTORATION IN DUCHENNE MUSCULAR DYSTROPHY PATIENTS AFTER SYSTEMIC PHOSPHORODIAMIDATE MORPHOLINO OLIGOMER TREATMENT

Molecular Mechanism of the E99K Mutation in Cardiac Actin (ACTC Gene) That Causes Apical Hypertrophy in Man and Mouse
Journal Article
Song, W., Dyer, E., Stuckey, D. J., Copeland, O., Leung, M. C., Bayliss, C., Messer, A., Wilkinson, R., Tremoleda, J. L., Schneider, M. D., Harding, S. E., Redwood, C. S., Clarke, K., Nowak, K., Monserrat, L., Wells, D. J., & Marston, S. B. (in press). Molecular Mechanism of the E99K Mutation in Cardiac Actin (ACTC Gene) That Causes Apical Hypertrophy in Man and Mouse. Journal of Biological Chemistry, 286(31), 27582–27593. https://doi.org/10.1074/jbc.M111.252320