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LONP-1 aids propagation of deleterious mtDNA

The integrity of the mitochondrial genome (mitochondrial DNA) is crucial for energy synthesis in all complex life. A study now reveals a vicious cycle of mtDNA replication within dysfunctional mitochondria that preferentially propagates genomes carrying deleterious mutations.

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Fig. 1: LONP-1 promotes the preferential replication of mutant mtDNAs (∆mtDNA) by ATFS-1 and POLG.


  1. Hahn, A. & Zuryn, S. Trends Cell Biol. 29, 227–240 (2019).

    Article  CAS  Google Scholar 

  2. Lin, Y. F. et al. Nature 533, 416–419 (2016).

    Article  CAS  Google Scholar 

  3. Gitschlag, B. L. et al. Cell Metab. 24, 91–103 (2016).

    Article  CAS  Google Scholar 

  4. Yang, Q. et al. Nat. Cell Biol. (2022).

  5. Fiorese, C. J. et al. Curr. Biol. 26, 2037–2043 (2016).

    Article  CAS  Google Scholar 

  6. Nargund, A. M., Pellegrino, M. W., Fiorese, C. J., Baker, B. M. & Haynes, C. M. Science 337, 587–590 (2012).

    Article  CAS  Google Scholar 

  7. Ahier, A. et al. Nat. Cell Biol. 20, 352–360 (2018).

    Article  CAS  Google Scholar 

  8. Bruno, C. et al. Am. J. Hum. Genet. 65, 611–620 (1999).

    Article  CAS  Google Scholar 

  9. Moraes, C. T. et al. N. Engl. J. Med. 320, 1293–1299 (1989).

    Article  CAS  Google Scholar 

  10. Suen, D. F., Narendra, D. P., Tanaka, A., Manfredi, G. & Youle, R. J. Proc. Natl Acad. Sci. USA 107, 11835–11840 (2010).

    Article  CAS  Google Scholar 

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Correspondence to Steven Zuryn.

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Kirmes, I., Zuryn, S. LONP-1 aids propagation of deleterious mtDNA. Nat Cell Biol 24, 127–128 (2022).

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