Messenger RNA

Translation (biology)

ATF4

Transcription factor

Biology

Gene

Endoplasmic reticulum

Unfolded protein response

Retrograde signaling

Caenorhabditis elegans

Integrated stress response

Signal transduction

Proteostasis

Mitochondrion

Cell biology

Genetics

Trends in Cell Biology

Eukaryotic cells must accurately monitor the integrity of the mitochondrial network to overcome environmental insults and respond to physiological cues. The mitochondrial unfolded protein response (UPRmt) is a mitochondrial-to-nuclear signaling pathway that maintains mitochondrial proteostasis, mediates signaling between tissues, and regulates organismal aging. Aberrant UPRmt signaling is associated with a wide spectrum of disorders, including congenital diseases as well as cancers and neurodegenerative diseases.

Folding the Mitochondrial UPR into the Integrated Stress Response