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Chapman J, Ng YS, Nicholls TJ. The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes. Life (Basel). 2020;10(9)doi: 10.3390/life10090164.
Chapman, J., Ng, Y. S., & Nicholls, T. J. (2020). The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes. Life (Basel, Switzerland), 10(9), . https://doi.org/10.3390/life10090164
Chapman, James, et al. "The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes." Life (Basel, Switzerland) vol. 10,9 (2020). doi: https://doi.org/10.3390/life10090164
Chapman J, Ng YS, Nicholls TJ. The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes. Life (Basel). 2020 Aug 26;10(9). doi: 10.3390/life10090164. PMID: 32858900; PMCID: PMC7555930.
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Life (Basel). 2020 Aug 26;10(9). doi: 10.3390/life10090164.

The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes.

Life (Basel, Switzerland)

James Chapman, Yi Shiau Ng, Thomas J Nicholls

Affiliations

  1. Wellcome Centre for Mitochondrial Research, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UK.
  2. Biosciences Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UK.
  3. Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UK.

PMID: 32858900 PMCID: PMC7555930 DOI: 10.3390/life10090164

Abstract

Mitochondria are complex organelles that harbour their own genome. Mitochondrial DNA (mtDNA) exists in the form of a circular double-stranded DNA molecule that must be replicated, segregated and distributed around the mitochondrial network. Human cells typically possess between a few hundred and several thousand copies of the mitochondrial genome, located within the mitochondrial matrix in close association with the cristae ultrastructure. The organisation of mtDNA around the mitochondrial network requires mitochondria to be dynamic and undergo both fission and fusion events in coordination with the modulation of cristae architecture. The dysregulation of these processes has profound effects upon mtDNA replication, manifesting as a loss of mtDNA integrity and copy number, and upon the subsequent distribution of mtDNA around the mitochondrial network. Mutations within genes involved in mitochondrial dynamics or cristae modulation cause a wide range of neurological disorders frequently associated with defects in mtDNA maintenance. This review aims to provide an understanding of the biological mechanisms that link mitochondrial dynamics and mtDNA integrity, as well as examine the interplay that occurs between mtDNA, mitochondrial dynamics and cristae structure.

Keywords: cristae; mitochondria; mitochondrial diseas; mitochondrial fission; mitochondrial fusion; mtDNA

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