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

Yi-Fan Lin, Anna M. Schulz, Mark W. Pellegrino, Yun Lu, Shai Shaham and Cole M. Haynes ()
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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

Nature, 2016, vol. 533, issue 7603, 416-419

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.

Date: 2016
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DOI: 10.1038/nature17989

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