Removal of damaged mitochondria helps maintain protein balance in cells to protect heart function

Mutations in two protein quality control pathway proteins are associated with cardiomyopathy in humans.

• CRYAB-R120G and BAG3-P209L mutations induce the formation of protein aggregates.
• Protein aggregates accumulate in cardiomyocytes, potentially disrupting sarcomere structure and function.
• Yeast models show that cytosolic protein aggregates can be taken up by mitochondria.
• Mitochondrial uptake of these aggregates may aid in their removal through a process called mitophagy.
• Mitophagy could play a role in maintaining cytosolic protein quality control in cardiomyocytes.

Simplified