Skeletal Muscle Mitochondrial and Autophagic Dysregulation Are Modifiable in Spinal Muscular Atrophy
Abnormal skeletal muscle mitochondrial turnover and dynamics are associated with disease progression in Smn<sup>2B/-</sup> mice despite compensatory elevations in upstream factors important for organelle synthesis and recycling. Exercise and UA enhance mitochondrial health in skeletal muscle, which indicates that lifestyle-based and pharmacological interventions may be effective countermeasures targeting the organelle for therapeutic remodelling in SMA.
Authors
- Andrew I. Mikhail (ORCID: https://orcid.org/0000-0001-8977-2055)
- Rashmi Kothary (ORCID: https://orcid.org/0000-0002-9239-7310)
- Joshua P. Nederveen
- Marc‐Olivier Deguise (ORCID: https://orcid.org/0000-0002-3802-1195)
- Mark A. Tarnopolsky (ORCID: https://orcid.org/0000-0003-0312-3746)
- Sean Y. Ng (ORCID: https://orcid.org/0000-0002-9238-3904)
- Melanie Lesinski (ORCID: https://orcid.org/0000-0002-5945-2998)
- Vladimir Ljubicic (ORCID: https://orcid.org/0000-0002-4592-4093)
- Yves De Repentigny (ORCID: https://orcid.org/0000-0001-8408-2442)
- Donald Xhuti
- Jennifer Chhor
Institutions
- University of Ottawa (CA)
- Children's Hospital of Eastern Ontario (CA)
- Ottawa Hospital (CA)
- McMaster University Medical Centre (CA)
- Ottawa Hospital Research Institute (CA)
- McMaster University (CA)
Publication Details
- Journal
- Journal of Cachexia Sarcopenia and Muscle
- Published
- 2025-02-01
- DOI
- https://doi.org/10.1002/jcsm.13701
- Citations
- 5
- Primary Topic
- Neurogenetic and Muscular Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 8.13
Funders
- Muscular Dystrophy Canada
- Canada Research Chairs
- Ministero dello Sviluppo Economico
- Ontario Ministry of Economic Development, Job Creation and Trade
- Canadian Institutes of Health Research
- Natural Sciences and Engineering Research Council of Canada