Metformin rescues muscle function in BAG3 myofibrillar myopathy models
Abstract
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 transgenic BAG3P209L-expressing fish...
Paper Details
Title
Metformin rescues muscle function in BAG3 myofibrillar myopathy models
Published Date
Oct 19, 2020
Journal
Volume
17
Issue
9
Pages
2494 - 2510
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History