Regulation of cellular proteostasis via mitochondrial protein import

Abstract

Mitochondria are the powerhouse of the cell, playing vital roles in energy production and metabolism. Most mitochondrial proteins are encoded in the nuclear DNA and must be synthesized in the cytosol before being transported into the appropriate mitochondrial compartments. Mitochondrial protein import is not only essential for mitochondrial biogenesis but also a crucial regulatory step in mitochondrial proteostasis surveillance and stress response. Additionally, defects in mitochondrial protein import lead to mislocalization of precursor proteins to the cytosol, disrupting cytosolic proteostasis and contributing to various human diseases. This review summarizes recent findings demonstrating that mitochondrial protein import is a key regulator of cellular proteostasis.

This article is part of the Theo Murphy meeting issue ‘ProteostaSys: a systems view of proteostasis’.