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Article

Zebrafish models of BAG3 myofibrillar myopathy suggest a toxic gain of function leading to BAG3 insufficiency.

Acta neuropathologica - 1 Dec 2014

Ruparelia Avnika A, Oorschot Viola, Vaz Raquel, Ramm Georg, Bryson-Richardson Robert J

Abstract excerpt

Mutations in the co-chaperone Bcl2-associated athanogene 3 (BAG3) can cause myofibrillar myopathy (MFM), a childhood-onset progressive muscle disease, characterized by the formation of protein aggregates and myofibrillar disintegration. In contrast to other MFM-causing proteins, BAG3 has no direct structural role, but regulates autophagy and the degradation of misfolded proteins. To investigate the mechanism of...

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Source

Identifiers

PMID: 25273835
DOI: 10.1007/s00401-014-1344-5

Genes

BAG3

Variants

Provenance

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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