Journal article

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

Rebecca A Ellwood, Jennifer E Hewitt, Roberta Torregrossa, Ashleigh M Philp, Justin P Hardee, Samantha Hughes, David van de Klashorst, Nima Gharahdaghi, Taslim Anupom, Luke Slade, Colleen S Deane, Michael Cooke, Timothy Etheridge, Mathew Piasecki, Adam Antebi, Gordon S Lynch, Andrew Philp, Siva A Vanapalli, Matthew Whiteman, Nathaniel J Szewczyk



Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive muscle degeneration and weakness due to mutations in the dystrophin gene. The symptoms of DMD share similarities with those of accelerated aging. Recently, hydrogen sulfide (H2S) supplementation has been suggested to modulate the effects of age-related decline in muscle function, and metabolic H2S deficiencies have been implicated in affecting muscle mass in conditions such as phenylketonuria. We therefore evaluated the use of sodium GYY4137 (NaGYY), a H2S-releasing molecule, as a possible approach for DMD treatment. Using the dys-1(eg33) Caenorhabditis elegans DMD model, we found that NaGYY trea..

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Awarded by Medical Research Council (MRC)

Awarded by NASA

Awarded by Biotechnology and Biological Sciences Research Council

Awarded by MRC

Awarded by United Mitochondrial Disease Foundation

Awarded by MRC Versus Arthritis Centre for Musculoskeletal Ageing Research Grants

Awarded by National Health and Medical Research Council

Funding Acknowledgements

R.A.E. was supported by the University of Nottingham School of Medicine. J.E.H. was supported by the Fulbright U.S. Student Program and the Germanistic Society of America. C.S.D. was supported by Medical Research Council (MRC) Grant MR/T026014/1. S.A.V. was supported by NASA Grant NNX15AL16G. This work was partially supported by Biotechnology and Biological Sciences Research Council Grant BB/N015894/1, MRC Grant MR/5002626/1 (to M.W.), United Mitochondrial Disease Foundation Grant PI -19-0985 (to M.W. and T.E.), the Brian Ridge Scholarship (R.T.), and the University of Exeter Diamond Jubilee Scholarship (L.S.). This research was also supported by MRC Versus Arthritis Centre for Musculoskeletal Ageing Research Grants MR/P021220/1 and MR/R502364/1 and National Institute for Health Research (NIHR) Nottingham Biomedical Research Centre. A.M.P. was supported by a Graham Painton Foundation Fellowship from University of New South Wales Sydney. J.P.H. was supported by a McKenzie Research Fellowship from The University of Melbourne. This work was supported by National Health and Medical Research Council Grant GNT1124474 (to G.S.L.). A.P. was supported by the Al and Val Rosenstrauss Fellowship from the Rebecca L. Cooper Medical Research Foundation. N.J.S. was supported by the Osteopathic Heritage Foundation. The views expressed are those of the author(s) and not necessarily those of the National Health Service, the NIHR, or the Department of Health and Social Care.