UMass Chan Medical School Faculty Publications

UMMS Affiliation

UMass Metabolic Network; Department of Molecular, Cell, and Cancer Biology

Publication Date


Document Type



Amino Acids, Peptides, and Proteins | Cell Biology | Cellular and Molecular Physiology | Genetic Phenomena | Molecular Biology


The mitochondrial network is not only required for the production of energy, essential cofactors and amino acids, but also serves as a signaling hub for innate immune and apoptotic pathways. Multiple mechanisms have evolved to identify and combat mitochondrial dysfunction to maintain the health of the organism. One such pathway is the mitochondrial unfolded protein response (UPR(mt)), which is regulated by the mitochondrial import efficiency of the transcription factor ATFS-1 in C. elegans and potentially orthologous transcription factors in mammals (ATF4, ATF5, CHOP). Upon mitochondrial dysfunction, import of ATFS-1 into mitochondria is reduced, allowing it to be trafficked to the nucleus where it promotes the expression of genes that promote survival and recovery of the mitochondrial network. Here, we discuss recent findings underlying UPR(mt) signal transduction and how this adaptive transcriptional response may interact with other mitochondrial stress response pathways.


mitochondrial UPR, ISR, stress response, proteostasis

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DOI of Published Version



Cell Res. 2018 Mar;28(3):281-295. doi: 10.1038/cr.2018.16. Epub 2018 Feb 9. Link to article on publisher's site

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Link to Article in PubMed

Journal/Book/Conference Title

Cell research

PubMed ID


Creative Commons License

Creative Commons Attribution 4.0 License
This work is licensed under a Creative Commons Attribution 4.0 License.