Article

Metformin rescues muscle function in BAG3 myofibrillar myopathy models

2019-03-12

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 identify therapies we generated two zebrafish models, one conditionally expressing BAG3 P209L and one with a nonsense mutation in bag3 . Whilst transgenic BAG3 P209L expressing fish display protein aggregation, modellin...

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Literature Corpus work: b15282e5-7ea4-5821-a680-39d2394a71eb
DOI: 10.1101/574806

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