Research ArticleMuscular Dystrophy

Urolithin A improves muscle function by inducing mitophagy in muscular dystrophy

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Science Translational Medicine  07 Apr 2021:
Vol. 13, Issue 588, eabb0319
DOI: 10.1126/scitranslmed.abb0319

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Mitigating myopathy through mitophagy

Mitochondrial dysfunction has been implicated in Duchenne muscular dystrophy (DMD). Luan and colleagues studied the role of mitophagy (mitochondrial autophagy) in muscle stem cells and DMD. Mitophagy was reduced in muscle biopsies from patients with muscular dystrophies and in mouse and worm models of DMD. Urolithin A, a mitophagy activator, up-regulated mitophagy-related genes and improved mitochondrial respiration, muscle stem cell regeneration and muscle function in models of DMD. This demonstrates how enhancing mitophagy could potentially help treat DMD.

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