2018
DOI: 10.1242/dmm.036137
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Muscle strength deficiency and mitochondrial dysfunction in a muscular dystrophy model of C. elegans and its functional response to drugs

Abstract: Muscle strength is a key clinical parameter used to monitor the progression of human muscular dystrophies, including Duchenne and Becker muscular dystrophies. Although Caenorhabditis elegans is an established genetic model for studying the mechanisms and treatments of muscular dystrophies, analogous strength-based measurements in this disease model are lacking. Here, we describe the first demonstration of the direct measurement of muscular strength in dystrophin-deficient C. elegans mutants using a micropillar… Show more

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Cited by 45 publications
(77 citation statements)
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“…These included increased cytosolic calcium, early onset, loss of mobility, mitochondrial damage, contractile machinery failure, muscle death, and shortened life span. This work complements recent studies with dystrophic worms demonstrating muscle weakness that is improved by drug treatments currently used to treat the disease (24), consolidating C. elegans as one of the most complete DMD animal models.…”
Section: Discussionsupporting
confidence: 67%
See 1 more Smart Citation
“…These included increased cytosolic calcium, early onset, loss of mobility, mitochondrial damage, contractile machinery failure, muscle death, and shortened life span. This work complements recent studies with dystrophic worms demonstrating muscle weakness that is improved by drug treatments currently used to treat the disease (24), consolidating C. elegans as one of the most complete DMD animal models.…”
Section: Discussionsupporting
confidence: 67%
“…C. elegans worms that genetically model DMD through loss-of-function mutations in dystrophin homolog (dys-1) are hyperactive and hypercontracted (23). Like DMD patients, dys-1 mutant worms display muscle weakness, which improves with prednisone and melatonin treatments (24). Under standard growth conditions (crawling on agar plates), dys-1 mutants display modest muscular and mitochondrial degeneration.…”
Section: Significancementioning
confidence: 99%
“…S2A). Similar to our findings, dys-1(eg33) worms, but not dys-1(cx18) worms were found to have significantly weaker adult muscle strength compared to wild-type worms (Hewitt et al . 2018).…”
Section: Resultssupporting
confidence: 91%
“…Aberrant mitochondrial activity has been previously reported in patients and animal models with DMD [8, 32-36], even prior to dystrophin assembly at the sarcolemma [31]. In this context, our results validate these studies and highlight an important signaling role for dys-1 , which evidently functions outside of its accepted role as a scaffolding protein in the DGC.…”
Section: Discussionsupporting
confidence: 88%