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Duchenne muscular dystrophy has received the majority of attention, whereby most discoveries suggest mitochondrial ATP synthesis may be reduced. However, not all studies support this finding. Furthermore, some studies have reported increased mitochondrial reactive oxygen species and propensity for permeability transition pore formation as an inducer of apoptosis, although divergent findings have also been described. A closer examination of the literature suggests the degree and direction of mitochondrial stress responses may depend on the progression of the disease, the muscle type examined, the mouse model used with regard to preclinical research, the precise metabolic pathways in consideration, and in some cases, the in vitro technique used to assess a given mitochondrial bioenergetic function. One intent of this review is to provide careful considerations for future experimental designs to resolve the heterogeneous nature of mitochondrial stress during the progression of Duchenne muscular dystrophy. Such considerations have implications for other muscular dystrophies as well which are addressed briefly herein. A renewed perspective of the term \u201cmitochondrial dysfunction\u201d is presented whereby stress responses might be re-explored in future investigations as direct contributors to myopathy versus an adaptive \u201creprogramming\u201d intended to maintain homeostasis in the face of disease stressors themselves. In so doing, the prospective development of mitochondrial enhancement therapies can be driven by advances in perspectives as much as experimental approaches when resolving the precise relationships between mitochondrial remodeling and muscle weakness in Duchenne and, indeed, other muscular dystrophies. <\/jats:p>","DOI":"10.1152\/ajpcell.00249.2022","type":"journal-article","created":{"date-parts":[[2022,7,11]],"date-time":"2022-07-11T19:23:56Z","timestamp":1657567436000},"page":"C718-C730","update-policy":"https:\/\/doi.org\/10.1152\/aps-crossmark-policy","source":"Crossref","is-referenced-by-count":41,"title":["Mitochondrial stress responses in Duchenne muscular dystrophy: metabolic dysfunction or adaptive reprogramming?"],"prefix":"10.1152","volume":"323","author":[{"given":"Catherine A.","family":"Bellissimo","sequence":"first","affiliation":[{"name":"School of Kinesiology and Health Science, Muscle Health Research Centre, York University, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madison C.","family":"Garibotti","sequence":"additional","affiliation":[{"name":"School of Kinesiology and Health Science, Muscle Health Research Centre, York University, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8087-9021","authenticated-orcid":false,"given":"Christopher G. 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