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Mitochondrial (mt) DNA encodes factors essential for cellular respiration, therefore its level and integrity are crucial.  ABF 2 encodes a mitochondrial DNA-binding protein and its null mutation ( Δabf2 ) induces mtDNA instability in  Saccharomyces cerevisiae . Mhr1 is a mitochondrial recombinase that mediates the predominant form of mtDNA replication and acts in mtDNA segregation and the repair of mtDNA double-stranded breaks (DSBs). However, the involvement of Mhr1 in prevention of mtDNA deletion mutagenesis is unknown. In this study we used  Δabf2 mhr1-1  double-mutant cells, which lose mitochondrial function in media containing fermentable carbon sources, to investigate whether Mhr1 is a suppressor of mtDNA deletion mutagenesis. We used a suppresivity assay and Southern blot analysis to reveal that the  Δabf2  mutation causes mtDNA deletions rather than an mtDNA-lacking (ρ 0 ) phenotype, and observed that mtDNA deletions are exacerbated by an additional  mhr1-1  mutation. Loss of respiratory function due to mtDNA fragmentation occurred in ∆ mhr1  and ∆ abf2 mhr1-1  cells. However, exogenous introduction of Mhr1 into  Δabf2 mhr1-1  cells significantly rescued respiratory growth, suggesting that Mhr1-driven homologous mtDNA recombination prevents mtDNA instability.

Prevention of mitochondrial genomic instability in yeast by the mitochondrial recombinase Mhr1