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Current Medical Research and Opinion Volume 37, 2021 - Issue 3
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Neuromuscular Disease

Adenylosuccinic acid: a novel inducer of the cytoprotectant Nrf2 with efficacy in Duchenne muscular dystrophy

Emma Rybalkaa Institute for Health and Sport, Victoria University, Melbourne, Australia;b Australian Institute for Musculoskeletal Science, Victoria University, St Albans, AustraliaCorrespondence[email protected]
ORCID Iconhttps://orcid.org/0000-0002-4854-0036
ORCID Icon,
Craig A. Goodmanb Australian Institute for Musculoskeletal Science, Victoria University, St Albans, Australia;c Center for Muscle Research, Department of Physiology, The University of Melbourne, Parkville, AustraliaORCID Iconhttps://orcid.org/0000-0002-5874-7743
ORCID Icon,
Dean G. Campelja Institute for Health and Sport, Victoria University, Melbourne, Australia;b Australian Institute for Musculoskeletal Science, Victoria University, St Albans, Australia
,
Alan Hayesa Institute for Health and Sport, Victoria University, Melbourne, Australia;b Australian Institute for Musculoskeletal Science, Victoria University, St Albans, Australia;d Department of Medicine, Western Health, Melbourne Medical School, The University of Melbourne, Melbourne, AustraliaORCID Iconhttps://orcid.org/0000-0003-1398-3694
ORCID Icon &
Cara A. Timpania Institute for Health and Sport, Victoria University, Melbourne, Australia;b Australian Institute for Musculoskeletal Science, Victoria University, St Albans, AustraliaORCID Iconhttps://orcid.org/0000-0003-4567-4319
ORCID Icon
Pages 465-467 | Received 15 Aug 2020, Accepted 09 Dec 2020, Published online: 29 Jan 2021

References

  • Timpani CA, Goodman CA, Stathis CG, et al. Adenylosuccinic acid therapy ameliorates murine Duchenne Muscular Dystrophy. Sci Rep. 2020;10(1):1–18.
  • Bonsett C, Rudman A. The dystrophin connection—ATP? Med Hypotheses. 1992;38(2):139–154.
  • Agboola F, Lin GA, Fluetsch N, et al. The effectiveness and value of Deflazacort and Exon-Skipping therapies for the management of Duchenne muscular dystrophy: a summary from the Institute for Clinical and Economic Review’s New England Comparative Effectiveness Public Advisory Council. J Manag Care Spec Pharm. 2020;26(4):361–366.
  • Timpani CA, Hayes A, Rybalka E. Revisiting the dystrophin–ATP connection: how half a century of research still implicates mitochondrial dysfunction in Duchenne muscular dystrophy aetiology. Med Hypotheses. 2015;85(6):1021–1033.
  • Kourakis S, Timpani CA, de Haan JB, et al. Targeting Nrf2 for the treatment of Duchenne Muscular Dystrophy. Redox Biol. 2021;38:101803.
  • Petrillo S, Pelosi L, Piemonte F, et al. Oxidative stress in Duchenne muscular dystrophy: focus on the NRF2 redox pathway. Hum Mol Genet. 2017;26(14):2781–2790.
  • Safdar A, deBeer J, Tarnopolsky MA. Dysfunctional Nrf2-Keap1 redox signaling in skeletal muscle of the sedentary old. Free Radic Biol Med. 2010;49(10):1487–1493.
  • Huang D-D, Fan S-D, Chen X-Y, et al. Nrf2 deficiency exacerbates frailty and sarcopenia by impairing skeletal muscle mitochondrial biogenesis and dynamics in an age-dependent manner. Exp Gerontol. 2019;119:61–73.

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