Journal
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
Volume 63, Issue -, Pages 21-24Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.biocel.2015.01.023
Keywords
Mitochondrial DNA; Mitochondrial DNA mutations; Clonal expansion; Mitophagy; Nucleoids
Categories
Funding
- Marie Curie Project: Mitochondrial European Educational Training (MEET) MEET
- Futuro in Ricerca-FIRB
- Programmi di Ricerca Scientifica di Rilevante Interesse Nazionale (PRIN) PRIN
- Telethon Grants [GGP06233, GPP10005, GGP11182]
- Emilia-Romagna regional program ER-MITO
- Fondazione Galletti
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Mitochondria are cytoplasmic organelles containing their own multi-copy genome. They are organized in a highly dynamic network, resulting from balance between fission and fusion, which maintains homeostasis of mitochondrial mass through mitochondrial biogenesis and mitophagy. Mitochondrial DNA (mtDNA) mutates much faster than nuclear DNA. In particular, mtDNA point mutations and deletions may occur somatically and accumulate with aging, coexisting with the wild type, a condition known as heteroplasmy. Under specific circumstances, clonal expansion of mutant mtDNA may occur within single cells, causing a wide range of severe human diseases when mutant overcomes wild type. Furthermore, mtDNA deletions accumulate and clonally expand as a consequence of deleterious mutations in nuclear genes involved in mtDNA replication and maintenance, as well as in mitochondrial fusion genes (mitofusin-2 and OPA1), possibly implicating mtDNA nucleoids segregation. We here discuss how the intricacies of mitochondrial homeostasis impinge on the intracellular propagation of mutant mtDNA. This article is part of a Directed Issue entitled: Energy Metabolism Disorders and Therapies. (C) 2015 Elsevier Ltd. All rights reserved.
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