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Article

BAG3P215L/KO Mice as a Model of BAG3P209L Myofibrillar Myopathy.

The American journal of pathology - 1 Mar 2020

Robertson Rebecca, Conte Talita C, Dicaire Marie-Josée, Rymar Vladimir V, Sadikot Abbas F, Bryson-Richardson Robert J, Lavoie Josée N, O'Ferrall Erin, Young Jason C, Brais Bernard

Abstract excerpt

BCL-2-associated athanogene 3 (BAG3) is a co-chaperone to heat shock proteins important in degrading misfolded proteins through chaperone-assisted selective autophagy. The recurrent dominant BAG3-P209L mutation results in a severe childhood-onset myofibrillar myopathy (MFM) associated with progressive muscle weakness, cardiomyopathy, and respiratory failure. Because a homozygous knock-in (KI) strain for the...

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PMID: 31953038
DOI: 10.1016/j.ajpath.2019.11.005

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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