Document Detail

Why mitochondria must fuse to maintain their genome integrity.
MedLine Citation:
PMID:  23350575     Owner:  NLM     Status:  MEDLINE    
SIGNIFICANCE: The maintenance of mitochondrial genome integrity is a major challenge for cells to sustain energy production by respiration.
RECENT ADVANCES: Recently, mitochondrial membrane dynamics emerged as a key process contributing to prevent mitochondrial DNA (mtDNA) alterations. Indeed, both fundamental and clinical data suggest that disruption of mitochondrial fusion, related to mutations in the OPA1, MFN2, PINK1, and PARK2 genes, leads to the accumulation of mutations in the mitochondrial genome.
CRITICAL ISSUES: We discuss here the possibility that mitochondrial fusion acts as a direct mechanism to prevent the generation of altered mtDNA and to eliminate mutated deleterious genomes either by trans-complementation or by mitophagy.
FUTURE DIRECTIONS: Finally, we conclude this review with a short evolutionary comparison between the mechanisms involved in mitochondrial and bacterial modes of genome distribution and plasticity, highlighting possible common conserved processes required for the maintenance of their genome integrity, which should inspire our future investigations.
Sara Vidoni; Claudia Zanna; Michela Rugolo; Emmanuelle Sarzi; Guy Lenaers
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review     Date:  2013-03-28
Journal Detail:
Title:  Antioxidants & redox signaling     Volume:  19     ISSN:  1557-7716     ISO Abbreviation:  Antioxid. Redox Signal.     Publication Date:  2013 Aug 
Date Detail:
Created Date:  2013-07-01     Completed Date:  2014-01-31     Revised Date:  2014-08-04    
Medline Journal Info:
Nlm Unique ID:  100888899     Medline TA:  Antioxid Redox Signal     Country:  United States    
Other Details:
Languages:  eng     Pagination:  379-88     Citation Subset:  IM    
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MeSH Terms
DNA, Mitochondrial / genetics
Genome, Mitochondrial / genetics
Mitochondria / genetics*,  metabolism*
Mitochondrial Dynamics / genetics
Grant Support
Reg. No./Substance:
0/DNA, Mitochondrial

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