EP 5: Journal Club: HSPB8 frameshift mutant aggregates weaken chaperone-assisted selective autophagy in neuromyopathies
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EP 5: Journal Club: HSPB8 frameshift mutant aggregates weaken chaperone-assisted selective autophagy in neuromyopathies
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Episode 5 – Tedesco et al. 2023: Molecular Insights into HSPB8
In this episode, we dive into the 2023 study by Tedesco et al., titled "HSPB8 frameshift mutant aggregates weaken chaperone-assisted selective autophagy in neuromyopathies," published in Autophagy. The research reveals how frameshift mutations in the HSPB8 gene lead to toxic protein aggregation and disrupt critical protein quality control systems. These mutations result in an elongated C-terminal end of the HSPB8 protein, promoting aggregates that sequester CASA complex components like BAG3 and autophagy markers such as p62—ultimately impairing the cell’s ability to clear damaged proteins. We explore how this toxic mechanism contributes to muscle fiber damage. A must-listen for patients, families, and professionals seeking a clearer understanding of the disease and the importance of genetic testing.