Article
Metformin rescues muscle function in BAG3 myofibrillar myopathy models.
Autophagy - 1 Sept 2021
Ruparelia Avnika A, McKaige Emily A, Williams Caitlin, Schulze Keith E, Fuchs Margit, Oorschot Viola, Lacene Emmanuelle, Meregalli Mirella, Lee Clara, Serrano Rita J, Baxter Emily C, Monro Keyne, Torrente Yvan, Ramm Georg, Stojkovic Tanya, Lavoie Josée N, Bryson-Richardson Robert J
Abstract excerpt
Dominant de novo mutations in the co-chaperone BAG3 cause a severe form of myofibrillar myopathy, exhibiting progressive muscle weakness, muscle structural failure, and protein aggregation. To elucidate the mechanism of disease in, and identify therapies for, BAG3 myofibrillar myopathy, we generated two zebrafish models, one conditionally expressing BAG3P209L and one with a nonsense mutation in bag3. While...
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