Evaluating and responding to mitochondrial dysfunction: The mitochondrial unfolded-protein response and beyond Journal Article


Authors: Haynes, C. M.; Fiorese, C. J.; Lin, Y. F.
Article Title: Evaluating and responding to mitochondrial dysfunction: The mitochondrial unfolded-protein response and beyond
Abstract: During development and cellular differentiation, tissue- and cell-specific programs mediate mitochondrial biogenesis to meet physiological needs. However, environmental and disease-associated factors can perturb mitochondrial activities, requiring cells to adapt to protect mitochondria and maintain cellular homeostasis. Several mitochondrion-to-nucleus signaling pathways, or retrograde responses, have been described, but the mechanisms by which mitochondrial stress or dysfunction is sensed to coordinate precisely the appropriate response has only recently begun to be understood. Recent studies of the mitochondrial unfolded-protein response (UPRmt) indicate that the cell monitors mitochondrial protein import efficiency as an indicator of mitochondrial function. Here, we review how the cell evaluates mitochondrial function and regulates transcriptional induction of the UPRmt, adapts protein-synthesis rates and activates mitochondrial autophagy to promote mitochondrial function and cell survival during stress. © 2013 Elsevier Ltd.
Keywords: signal transduction; review; nonhuman; protein function; protein localization; cell survival; protein p53; protein synthesis; saccharomyces cerevisiae; abc transporter; autophagy; mitochondrial protein; cell stress; unfolded protein response; attenuation; mitochondrial stress; transcriptional responses; early response gene
Journal Title: Trends in Cell Biology
Volume: 23
Issue: 7
ISSN: 0962-8924
Publisher: Elsevier Inc.  
Date Published: 2013-07-01
Start Page: 311
End Page: 318
Language: English
DOI: 10.1016/j.tcb.2013.02.002
PROVIDER: scopus
PMCID: PMC3700555
PUBMED: 23489877
DOI/URL:
Notes: --- - "Export Date: 1 August 2013" - "CODEN: TCBIE" - "Source: Scopus"
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MSK Authors
  1. Cole Haynes
    24 Haynes
  2. Yi-Fan Lin
    4 Lin