All Outputs (65)

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

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.

Transgenic Overexpression of LARGE Induces alpha-Dystroglycan Hyperglycosylation in Skeletal and Cardiac Muscle (2010)
Journal Article
Brockington, M. A., Torelli, S., Sharp, P. S., Liu, K., Cirak, S., Brown, S. C., Wells, D. J., & Muntoni, F. (2010). Transgenic Overexpression of LARGE Induces alpha-Dystroglycan Hyperglycosylation in Skeletal and Cardiac Muscle. PLoS ONE, 5(12), e14434. https://doi.org/10.1371/journal.pone.0014434

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

In vivo fluorescence lifetime tomography of a FRET probe expressed in mouse
Journal Article
McGinty, J., Stuckey, D. W., Solovjev, V. Y., Wylezinska-Arridge, M., Wells, D. J., Arridge, S. R., P M W, F., Hajnal, J. V., & Sardini, A. In vivo fluorescence lifetime tomography of a FRET probe expressed in mouse. Biomedical Optics Express, 2(7), 1907-1917. https://doi.org/10.1364/BOE.2.001907

Chronic Systemic Therapy With Low-dose Morpholino Oligomers Ameliorates the Pathology and Normalizes Locomotor Behavior in mdx Mice
Journal Article
Malerba, A., Graham, I., Sharp, P., Popplewell, L., Foster, K., Wells, D. J., & Dickson, G. Chronic Systemic Therapy With Low-dose Morpholino Oligomers Ameliorates the Pathology and Normalizes Locomotor Behavior in mdx Mice. Molecular Therapy, 19(2), 345-54. https://doi.org/10.1038/mt.2010.261

Metformin Treatment Has No Beneficial Effect in a Dose-Response Survival Study in the SOD1(G93A) Mouse Model of ALS and Is Harmful in Female Mice
Journal Article
Kaneb, H. M., Sharp, P. S., Rahmani-Kondori, N., & Wells, D. J. Metformin Treatment Has No Beneficial Effect in a Dose-Response Survival Study in the SOD1(G93A) Mouse Model of ALS and Is Harmful in Female Mice. PLoS ONE, 6(9), e24189. https://doi.org/10.1371/journal.pone.0024189

Muscular dystrophies due to glycosylation defects: diagnosis and therapeutic strategies
Journal Article
Muntoni, F., Torelli, S., Wells, D. J., & Brown, S. C. Muscular dystrophies due to glycosylation defects: diagnosis and therapeutic strategies. Current Opinion in Neurology, 24(5), 437-442. https://doi.org/10.1097/WCO.0b013e32834a95e3

Heat shock protein 27 overexpression mitigates cytokine-induced islet apoptosis and streptozotocin-induced diabetes
Journal Article
Dai, T., Patel-Chamberlin, M., Natarajan, R., Todorov, I., Ma, J., LaPage, J., Phillips, L., Nast, C. C., Becerra, D., Chuang, P., Tong, L., De Belleroche, J., Wells, D. J., Wang, Y., & Adler, S. G. Heat shock protein 27 overexpression mitigates cytokine-induced islet apoptosis and streptozotocin-induced diabetes. Endocrinology, 150(7), 3031-9. https://doi.org/10.1210/en.2008-0732

A New Extensively Characterised Conditionally Immortal Muscle Cell-Line for Investigating Therapeutic Strategies in Muscular Dystrophies
Journal Article
Muses, S., Morgan, J. E., & Wells, D. J. A New Extensively Characterised Conditionally Immortal Muscle Cell-Line for Investigating Therapeutic Strategies in Muscular Dystrophies. PLoS ONE, 6(9), e24826. https://doi.org/10.1371/journal.pone.0024826

Restoration of dystrophin expression using the Sleeping Beauty transposon
Journal Article
Muses, S., Morgan, J. E., & Wells, D. J. Restoration of dystrophin expression using the Sleeping Beauty transposon. https://doi.org/10.1371/currents.RRN1296

Exon skipping and dystrophin restoration in patients with Duchenne muscular dystrophy after systemic phosphorodiamidate morpholino oligomer treatment: an open-label, phase 2, dose-esclation study
Journal Article
Cirak, S., Arechavala-Gomeza, V., Guglieri, M., Feng, L., Torelli, S., Anthony, K., Abbs, S., Garralda, M. E., Wells, D., Dickson, G., Wood, M. J. A., Wilton, S. D., Straub, V., Shrewsbury, S., Sewry, C., Morgan, J. E., Bushby, K., & Muntoni, F. Exon skipping and dystrophin restoration in patients with Duchenne muscular dystrophy after systemic phosphorodiamidate morpholino oligomer treatment: an open-label, phase 2, dose-esclation study. https://doi.org/10.1016/S0140-6736%2811%2960756-3

Heat shock protein 27 protects against atherogenesis via an Estrogen-Dependent Mechanism: Role of Selective Estrogen Receptor Beta Modulation
Journal Article
Rayner, K., Sun, J., Chen, Y. X., McNulty, M., Simard, T., Zhao, X., Wells, D. J., de Belleroche, J., & O'Brien, E. R. Heat shock protein 27 protects against atherogenesis via an Estrogen-Dependent Mechanism: Role of Selective Estrogen Receptor Beta Modulation. Arteriosclerosis, Thrombosis, and Vascular Biology, 29(11), 1751-6. https://doi.org/10.1161/ATVBAHA.109.193656

Physiological Characterization of muscle strength with variable levels of Dystrophin Restoration in mdx Mice following local antisense therapy
Journal Article
Sharp, P. S., Bye-a-Jee, H., & Wells, D. J. Physiological Characterization of muscle strength with variable levels of Dystrophin Restoration in mdx Mice following local antisense therapy. Molecular Therapy, 19(1), 165-171. https://doi.org/10.1038/mt.2010.213

Forster resonance energy transfer imaging in vivo with approximated radiative transfer equation
Journal Article
Soloviev, V. Y., McGinty, J., Stuckey, D. W., Laine, R., Wylezinska-Arridge, M., Wells, D. J., Sardini, A., Hajnal, J. V., French, P. M. W., & Arridge, S. R. Forster resonance energy transfer imaging in vivo with approximated radiative transfer equation. Applied Optics, 50(36), 6583-6590

Overexpression of heat shock protein 27 reduces cortical damage after cerebral ischemia
Journal Article
Van Der Weerd, L., Tariq Akbar, M., Aron Badin, R., Valentim, L. M., Thomas, D. L., Wells, D. J., Latchman, D. S., Gadian, D. G., Lythgoe, M. F., & De Belleroche, J. S. Overexpression of heat shock protein 27 reduces cortical damage after cerebral ischemia. https://doi.org/10.1038/jcbfm.2009.249

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

Electroporation and ultrasound enhanced non-viral gene delivery in vitro and in vivo
Journal Article
Wells, D. J. Electroporation and ultrasound enhanced non-viral gene delivery in vitro and in vivo. Cell Biology and Toxicology, 26(1), 21-28. https://doi.org/10.1007/s10565-009-9144-8