Bcl-2-associated athanogene 3 (BAG3) is a protein that serves as a co-chaperone to heat-shock proteins, inhibits apoptosis and facilitates the degradation of misfolded proteins. Mutations to BAG3, specifically the dominantly expressed BAG3P209L, can result in a severe childhood myofibrillar myopathy. Bag3 myofibrillar myopathy causes progressive muscle weakness with cardiomyopathy and respiratory failure developing in the second decade of life, eventually leading to death. Two mouse lines were developed to study this disease: a knock-out line, and a knock-in line with a mutation homologous to the P209L mutation seen in humans.

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