Measuring Clinical Effectiveness of Medicinal Products for the Treatment of Duchenne Muscular Dystrophy

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

doi:10.1016/j.nmd.2014.09.003

All Outputs (4)

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., …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)
Journal Article
Charles, J. P., Cappellari, O., Spence, A., Wells, D. J., & Hutchinson, J. R. (2015). DEVELOPING, TESTING AND OPTIMISING A MOUSE HINDLIMB MUSCULOSKELETAL MODEL. FASEB Journal, 29,

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., …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