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Maintenance and propagation of a deleterious mitochondrial genome by the mitochondrial unfolded protein response

Author

Listed:
  • Yi-Fan Lin

    (Cell Biology Program, Memorial Sloan Kettering Cancer Center)

  • Anna M. Schulz

    (Cell Biology Program, Memorial Sloan Kettering Cancer Center)

  • Mark W. Pellegrino

    (Cell Biology Program, Memorial Sloan Kettering Cancer Center)

  • Yun Lu

    (Laboratory of Developmental Genetics, The Rockefeller University)

  • Shai Shaham

    (Laboratory of Developmental Genetics, The Rockefeller University)

  • Cole M. Haynes

    (Cell Biology Program, Memorial Sloan Kettering Cancer Center
    BCMB Allied Program, Weill Cornell Medical College)

Abstract

In the context of mitochondrial genome heteroplasmy that causes defective oxidative phosphorylation in C. elegans, the ATFS-1-mediated mitochondrial unfolded protein response maintains the deleterious mitochondrial DNA in an attempt to recover oxidative phosphorylation activity and avoid cellular dysfunction.

Suggested Citation

  • Yi-Fan Lin & Anna M. Schulz & Mark W. Pellegrino & Yun Lu & Shai Shaham & Cole M. Haynes, 2016. "Maintenance and propagation of a deleterious mitochondrial genome by the mitochondrial unfolded protein response," Nature, Nature, vol. 533(7603), pages 416-419, May.
    • Handle: RePEc:nat:nature:v:533:y:2016:i:7603:d:10.1038_nature17989
    DOI: 10.1038/nature17989
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    Cited by:

    1. Evelyn Fessler & Luisa Krumwiede & Lucas T. Jae, 2022. "DELE1 tracks perturbed protein import and processing in human mitochondria," Nature Communications, Nature, vol. 13(1), pages 1-15, December.
    2. Bryan L. Gitschlag & Claudia V. Pereira & James P. Held & David M. McCandlish & Maulik R. Patel, 2024. "Multiple distinct evolutionary mechanisms govern the dynamics of selfish mitochondrial genomes in Caenorhabditis elegans," Nature Communications, Nature, vol. 15(1), pages 1-14, December.
    3. Eirini Lionaki & Ilias Gkikas & Ioanna Daskalaki & Maria-Konstantina Ioannidi & Maria I. Klapa & Nektarios Tavernarakis, 2022. "Mitochondrial protein import determines lifespan through metabolic reprogramming and de novo serine biosynthesis," Nature Communications, Nature, vol. 13(1), pages 1-16, December.
    4. Chih-Yao Chung & Kritarth Singh & Vassilios N. Kotiadis & Gabriel E. Valdebenito & Jee Hwan Ahn & Emilie Topley & Joycelyn Tan & William D. Andrews & Benoit Bilanges & Robert D. S. Pitceathly & Gyorgy, 2021. "Constitutive activation of the PI3K-Akt-mTORC1 pathway sustains the m.3243 A > G mtDNA mutation," Nature Communications, Nature, vol. 12(1), pages 1-16, December.

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