The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes
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 sev...
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| Format: | Article |
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MDPI AG
2020-08-01
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| Series: | Life |
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| Online Access: | https://www.mdpi.com/2075-1729/10/9/164 |
| _version_ | 1827707553022214144 |
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| author | James Chapman Yi Shiau Ng Thomas J. Nicholls |
| author_facet | James Chapman Yi Shiau Ng Thomas J. Nicholls |
| author_sort | James Chapman |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T16:49:10Z |
| format | Article |
| id | doaj.art-d90762fcf5b7469c8899ca2c36f6c2c0 |
| institution | Directory Open Access Journal |
| issn | 2075-1729 |
| language | English |
| last_indexed | 2024-03-10T16:49:10Z |
| publishDate | 2020-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj.art-d90762fcf5b7469c8899ca2c36f6c2c02023-11-20T11:24:09ZengMDPI AGLife2075-17292020-08-0110916410.3390/life10090164The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial MembranesJames Chapman0Yi Shiau Ng1Thomas J. Nicholls2Wellcome Centre for Mitochondrial Research, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UKWellcome Centre for Mitochondrial Research, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UKWellcome Centre for Mitochondrial Research, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UKMitochondria 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.https://www.mdpi.com/2075-1729/10/9/164mitochondriamtDNAcristaemitochondrial fissionmitochondrial fusionmitochondrial diseas |
| spellingShingle | James Chapman Yi Shiau Ng Thomas J. Nicholls The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes Life mitochondria mtDNA cristae mitochondrial fission mitochondrial fusion mitochondrial diseas |
| title | The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes |
| title_full | The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes |
| title_fullStr | The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes |
| title_full_unstemmed | The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes |
| title_short | The Maintenance of Mitochondrial DNA Integrity and Dynamics by Mitochondrial Membranes |
| title_sort | maintenance of mitochondrial dna integrity and dynamics by mitochondrial membranes |
| topic | mitochondria mtDNA cristae mitochondrial fission mitochondrial fusion mitochondrial diseas |
| url | https://www.mdpi.com/2075-1729/10/9/164 |
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