How do genome abnormalities impact mitochondrial homeostasis?

Abstract The genome of eukaryotes encodes thousands of proteins needed to ensure cell health. Hence, an imbalance in the genome has profound consequences for protein homeostasis (proteostasis). The genome and proteome are interlocked in a mutual relationship, which impacts the functions of cellular organelles like mitochondria. However, the underlying mechanisms remain an enigma. Here, we highlight this relationship and outline some open questions to be addressed.