Journal
ISCIENCE
Volume 23, Issue 5, Pages -Publisher
CELL PRESS
DOI: 10.1016/j.isci.2020.101087
Keywords
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Categories
Funding
- AriSLA through HyperALS project
- AFM-Telethon project [2018]
- AFMTelethon project [21021]
- National Health and Medical Research Council [1101085, 1185427]
- Scott Sullivan MND Research Fellowship (Queensland Brain Institute)
- Scott Sullivan MND Research Fellowship (Royal Brisbane & Women's Hospital Foundation)
- Scott Sullivan MND Research Fellowship (MND)
- Scott Sullivan MND Research Fellowship (Me Foundation)
- Australian Institute for Bioengineering and Nanotechnology
- National Health and Medical Research Council of Australia [1101085, 1185427] Funding Source: NHMRC
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Patients with ALS show, in addition to the loss of motor neurons in the spinal cord, brainstem, and cerebral cortex, an abnormal depletion of energy stores alongside hypermetabolism. In this study, we show that bioenergetic defects and muscle remodeling occur in skeletal muscle of the SOD1(G93A) mouse model of ALS mice prior to disease onset and before the activation of muscle denervation markers, respectively. These changes in muscle physiology were followed by an increase in energy expenditure unrelated to physical activity. Finally, chronic treatment of SOD1(G93A) mice with Ranolazine, an FDA-approved inhibitor of fatty acid beta-oxidation, led to a decrease in energy expenditure in symptomatic SOD1(G93A) mice, and this occurred in parallel with a robust, albeit temporary, recovery of the pathological phenotype.
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