Integrating the UPR^mt into the mitochondrial maintenance network

Abstract

Mitochondrial function is central to many different processes in the cell, from oxidative phosphorylation to the synthesis of iron–sulfur clusters. Therefore, mitochondrial dysfunction underlies a diverse array of diseases, from neurodegenerative diseases to cancer. Stress can be communicated to the cytosol and nucleus from the mitochondria through many different signals, and in response the cell can effect everything from transcriptional to post-transcriptional responses to protect the mitochondrial network. How these responses are coordinated have only recently begun to be understood. In this review, we explore how the cell maintains mitochondrial function, focusing on the mitochondrial unfolded protein response (UPR^mt), a transcriptional response that can activate a wide array of programs to repair and restore mitochondrial function.

Graphical Abstract

Keywords:

• Mitochondrial unfolded protein response
• mitochondrial dysfunction
• mitochondrial stress response
• integrated stress response
• mitochondria quality control

Disclosure statement

The authors report no declarations of interest.

Additional information

Funding

This work was supported by grants from the NIH (R01HL127891 and R01AG047182 to C.M.H.).