Dysfunction of cellular mechanisms maintaining proteostasis leads to accumulation of misfolded proteins and their aggregates, which often results in debilitating human diseases, such as neurodegenerative diseases (ND). Interestingly, those diseases are also frequently coupled with mitochondrial defects. Although proteostasis and mitochondria are individually studied intensively in the context of ND, the relationship between those two are poorly understood. Our recent study demonstrates that mitochondria directly participate in proteostasis through import and degradation of cytosolic proteins prone to aggregation and we term this pathway MAGIC (Mitochondria as Guardian in Cytosol). MAGIC is essential for timely clearance of protein aggregates after acute proteotoxic stress.

Rong Li, Ph.D.