Therapeutic Potential of Skeletal Muscle Plasticity and Slow Muscle Programming for Muscular Dystrophy

Grant number: 1124474 | Funding period: 2017 - 2021

Completed

Abstract

There is no cure for DMD, a devastating, life-limiting muscle disease causing progressive muscle wasting in boys and young men. A potential therapy may come from modulating muscle activity patterns to promote a protective slow muscle phenotype through low-frequency stimulation protocols and/or well-described pharmacological ‘exercise mimetics’. This proposal will evaluate their therapeutic merit in mouse models of DMD to answer the key questions to advance this approach to the clinic.

Related publications (8)

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Increasing muscle contractility through low-frequency stimulation alters tibial bone geometry and reduces bone strength in mdx and dko dystrophic mice

AS Chan, JP Hardee, M Blank, EHJ Cho, NE McGregor, NA Sims, GS Lynch

2023-07-01

Duchenne muscular dystrophy (DMD) is a severe muscle wasting disease caused by mutations or deletions in the dystrophin gene, for ..

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Dystrophin deficiency disrupts muscle clock expression and mitochondrial quality control in mdx mice

JP Hardee, MK Caldow, ASM Chan, SK Plenderleith, J Trieu, R Koopman, GS Lynch

2021-08-01

Impaired oxidative capacity and mitochondrial function contribute to the dystrophic pathology in muscles of patients with Duchenne..

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Bone Geometry Is Altered by Follistatin-Induced Muscle Growth in Young Adult Male Mice

ASM Chan, NE McGregor, IJ Poulton, JP Hardee, EHJ Cho, TJ Martin, P Gregorevic, NA Sims, GS Lynch

2021-04-01

The development of the musculoskeletal system and its maintenance depends on the reciprocal relationship between muscle and bone. ..

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Mitochondrial hydrogen sulfide supplementation improves health in the C. elegans Duchenne muscular dystrophy model

RA Ellwood, JE Hewitt, R Torregrossa, AM Philp, JP Hardee, S Hughes, D van de Klashorst, N Gharahdaghi, T Anupom, L Slade, CS Deane, M Cooke, T Etheridge, M Piasecki, A Antebi, GS Lynch, A Philp, SA Vanapalli, M Whiteman, NJ Szewczyk

2021-03-02

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive muscle degeneration and weakness ..

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Metabolic remodeling of dystrophic skeletal muscle reveals biological roles for dystrophin and utrophin in adaptation and plasticity

JP Hardee, KJB Martins, PM Miotto, JG Ryall, SM Gehrig, B Reljic, T Naim, JD Chung, J Trieu, K Swiderski, AM Philp, MJ Watt, DA Stroud, R Koopman, GR Steinberg, GS Lynch

2021-03-01

Objectives: Preferential damage to fast, glycolytic myofibers is common in many muscle-wasting diseases, including Duchenne muscul..

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Aging and Skeletal Muscle

Gordon S Lynch

2020-01-01

Some of the most serious consequences of aging are its effects on skeletal muscle particularly the progressive loss of mass and fu..

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Overcoming nature's paradox in skeletal muscle to optimise animal production

GS Lynch, R Koopman

2019-01-01

Nature's paradox in skeletal muscle describes the seemingly mutually exclusive relationship between muscle fibre size and oxidativ..

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Dietary meat and protection against sarcopenia

GS Lynch, R Koopman

2018-10-01

Sarcopenia describes the age-related loss of skeletal muscle mass and associated muscle weakness. Sarcopenia is a major global hea..

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