Abstract
Extensive complementation between fused mitochondria is indicated by recombination of 'parental' mitochondrial (mt) DNA (ref. 1,2) of yeast and plant cells. It has been difficult, however, to demonstrate the occurrence of complementation between fused mitochondria in mammalian species through the presence of recombinant mtDNA molecules3, because sequence of mtDNA throughout an individual tends to be uniform4 owing to its strictly maternal inheritance5,6,7. We isolated two types of respiration-deficient cell lines, with pathogenic mutations in mitochondrial tRNAIle or tRNALeu(UUR) genes from patients with mitochondrial diseases. The coexistence of their mitochondria within hybrids restored their normal morphology and respiratory enzyme activity by 10–14 days after fusion, indicating the presence of an extensive and continuous exchange of genetic contents between the mitochondria. This complementation between fused mitochondria may represent a defence of highly oxidative organelles against mitochondrial dysfunction caused by the accumulation of mtDNA lesions with age.
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Acknowledgements
This work was supported in part by a grant for a Research Fellowship from the Japan Society for Promotion of Science for Young Scientists to K.I., by a grant for the Hayashi project of TARA, University of Tsukuba, and by Grants-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology of Japan to J.-I.H.
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Ono, T., Isobe, K., Nakada, K. et al. Human cells are protected from mitochondrial dysfunction by complementation of DNA products in fused mitochondria. Nat Genet 28, 272–275 (2001). https://doi.org/10.1038/90116
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DOI: https://doi.org/10.1038/90116
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