The mitochondrial unfolded protein response: Signaling from the powerhouse

J Biol Chem. 2017 Aug 18;292(33):13500-13506. doi: 10.1074/jbc.R117.791061. Epub 2017 Jul 7.

Abstract

Mitochondria are multifaceted and indispensable organelles required for cell performance. Accordingly, dysfunction to mitochondria can result in cellular decline and possibly the onset of disease. Cells use a variety of means to recover mitochondria and restore homeostasis, including the activation of retrograde pathways such as the mitochondrial unfolded protein response (UPRmt). In this Minireview, we will discuss how cells adapt to mitochondrial stress through UPRmt regulation. Furthermore, we will explore the current repertoire of biological functions that are associated with this essential stress-response pathway.

Keywords: Mitochondrial dysfunction; cell metabolism; cell signaling; cellular regulation; mitochondria; mitochondrial quality control; mitochondrial stress signaling; mitochondrial unfolded protein response; stress response.

Publication types

  • Comparative Study
  • Review
  • Research Support, Non-U.S. Gov't
  • Research Support, N.I.H., Extramural

MeSH terms

  • Allostasis*
  • Animals
  • Endoplasmic Reticulum / enzymology
  • Endoplasmic Reticulum / metabolism
  • Endoplasmic Reticulum Stress
  • Genome, Mitochondrial
  • Hematopoietic Stem Cells / cytology
  • Hematopoietic Stem Cells / enzymology
  • Hematopoietic Stem Cells / metabolism
  • Humans
  • Immunity, Innate
  • Mitochondria / enzymology
  • Mitochondria / metabolism*
  • Models, Biological*
  • Signal Transduction*
  • Stress, Physiological*
  • Unfolded Protein Response*